☆ 4.6 Review

Valosin containing protein (VCP): initiator, modifier, and potential drug target for neurodegenerative diseases

MOLECULAR NEURODEGENERATION (2023)

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Interactome screening of C9orf72 dipeptide repeats reveals VCP sequestration and functional impairment by polyGA

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WIPI2 positively regulates mitophagy by promoting mitochondrial recruitment of VCP

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Summary: This study reveals the crucial role of WIPI2 in mitochondrial recruitment of VCP to promote outer mitochondrial membrane protein degradation and eventual mitophagy. WIPI2 is recruited to damaged mitochondria upon mitophagy induction and its loss impairs mitophagy induced by mitochondrial damaging agents. WIPI2 binds to and promotes the transportation of AAA-ATPase VCP/p97 to damaged mitochondria, and its depletion blunts the recruitment of VCP to damaged mitochondria, leading to reduction in degradation of outer mitochondrial membrane proteins and mitophagy. Furthermore, WIPI2 is implicated in cell fate decision as cells deficient in WIPI2 are largely resistant to cell death induced by mitochondrial damage.

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p97/UBXD1 Generate Ubiquitylated Proteins That Are Sequestered into Nuclear Envelope Herniations in Torsin-Deficient Cells

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Pathogenic variants of Valosin-containing protein induce lysosomal damage and transcriptional activation of autophagy regulators in neuronal cells

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Summary: In this study, the impact of VCP mutants on lysosomes was investigated, revealing that VCP mutants induce the formation of aberrant multilamellar organelles and alter lysosomal activity. The study also showed that wild type VCP can counteract lysosomal damage induced by trehalose or mutant SOD1, potentially shedding light on the pathogenesis of VCP-related diseases.

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VCP inhibition induces an unfolded protein response and apoptosis in human acute myeloid leukemia cells

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Summary: Acute myeloid leukemia (AML) is a heterogeneous malignancy characterized by the accumulation of undifferentiated white blood cells in the bone marrow. Inhibition of the molecular chaperone VCP with the inhibitor CB-5083 leads to accumulation of ubiquitylated proteins and triggers apoptosis through the unfolded protein response. This suggests that targeting VCP may be a therapeutic strategy for AML, especially when combined with other drugs. However, further investigation is needed to address the toxicity and development of resistance associated with VCP inhibitors in animal models and clinical trials.

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A comprehensive coverage insurance for cells: revealing links between ribosome collisions, stress responses and mRNA surveillance

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Environmental factors in Parkinson's disease: New insights into the molecular mechanisms

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Summary: Parkinson's disease is a chronic neurodegenerative disorder characterized by motor impairment, autonomic dysfunction, and psychological and cognitive changes. The disease is caused by a combination of genetic factors, environmental factors, and lifestyles. The pathological hallmarks are intracellular inclusions containing α-synuclein aggregates and the loss of dopaminergic neurons in the substantia nigra.

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Lysosomal dysfunction in neurodegeneration: emerging concepts and methods

Vinod Udayar et al.

Summary: The understanding of lysosomes has advanced significantly, and their importance in cell signaling, transcription, and metabolism is now recognized. Genes regulating lysosomal function are implicated in neurodegenerative diseases. Technological advances have deepened our understanding of lysosomes in neurodegeneration and led to the development of disease-specific models.

TRENDS IN NEUROSCIENCES (2022)

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A Potential Mechanism for Targeting Aggregates With Proteasomes and Disaggregases in Liquid Droplets

Emma Mee Hayes et al.

Summary: Insoluble protein deposits are a common feature of neurodegenerative disorders and dementia. The process of misfolded protein aggregation involves liquid-liquid phase separation (LLPS), and enzymes like the ubiquitin-proteasome system, VCP/p97, and HSP70 are involved in disassembling and degrading the aggregates. These enzymes can reversibly assemble into liquid droplets and enhance disassembly through increased local concentration within the droplets.

FRONTIERS IN AGING NEUROSCIENCE (2022)

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Article Genetics & Heredity

Monogenic Parkinson's Disease: Genotype, Phenotype, Pathophysiology, and Genetic Testing

Fangzhi Jia et al.

Summary: Parkinson's disease can be caused by a single pathogenic variant, but studying the genetic factors can provide important insights into the disease. This review discusses the different genetic forms, including their genotype, phenotype, pathophysiology, and geographic and ethnic distribution. It also highlights the influence of ethnicity and geography on monogenic Parkinson's disease and the need for global efforts to identify new candidate genes.

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Multisystem Proteinopathy Due to VCP Mutations: A Review of Clinical Heterogeneity and Genetic Diagnosis

Gerald Pfeffer et al.

Summary: This work reviews the clinical features and genetic diagnosis of diseases caused by mutations in the VCP gene. VCP is crucial for cellular functions and its mutations can lead to various neurodegenerative diseases and non-neurological symptoms.

GENES (2022)

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Article Endocrinology & Metabolism

Clinical Characteristics and Pathogenic Gene Identification in Chinese Patients With Paget's Disease of Bone

Xiaohui Tao et al.

Summary: This study evaluated the clinical features of sporadic Paget's disease of bone (PDB) in China and identified potential genetic abnormalities. The results showed that Chinese patients have an earlier onset age, more severe symptoms, and a lower frequency of SQSTM1 gene mutation compared to Western patients. Additionally, potential pathogenic genes and pathways related to neurodegeneration and ALS were identified.

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NGLY1 Deficiency, a Congenital Disorder of Deglycosylation: From Disease Gene Function to Pathophysiology

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Summary: N-Glycanase 1 (NGLY1) is a cytosolic enzyme involved in ERAD, and its role in animal development and physiology, as well as the pathophysiology of NGLY1 deficiency, has been intensively studied. Loss of NGLY1 affects critical processes such as proteasome bounce-back response, mitochondrial function, and BMP signaling.

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Genotype-phenotype correlations in valosin-containing protein disease: a retrospective muticentre study

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Summary: This study characterized patients with VCP gene mutations and investigated genotype-phenotype correlations, finding common symptoms and genetic variants. Specific genetic variants showed trends in affecting age of onset and severity. Additionally, the study provided valuable information on disease progression risk factors.

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Adaptor-Specific Antibody Fragment Inhibitors for the Intracellular Modulation of p97 (VCP) Protein-Protein Interactions

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Summary: Protein-protein interactions (PPIs) play crucial roles in cellular signaling and functions. Precise modulation of PPIs can help understand their roles in cellular events and be therapeutically valuable. In this study, an antibody fragment-based modulator for the PPI between p97 and its adaptor protein NSFL1C was developed, demonstrating its potential application in therapeutic interventions by disrupting specific intracellular PPIs.

JOURNAL OF THE AMERICAN CHEMICAL SOCIETY (2022)

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Review Neurosciences

The Cure VCP Scientific Conference 2021: Molecular and clinical insights into neurodegeneration and myopathy linked to multisystem proteinopathy-1 (MSP-1)

Michelle A. Johnson et al.

Summary: The 2021 VCP Scientific Conference, organized by Cure VCP Disease, Inc., brought together researchers and professionals in the field to discuss the physiological roles of VCP, disease mechanisms, and potential therapies. The conference also aimed to promote diversity and inclusion in VCP research.

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Compounds activating VCP D1 ATPase enhance both autophagic and proteasomal neurotoxic protein clearance

Lidia Wrobel et al.

Summary: Enhancing the removal of aggregate-prone toxic proteins is a rational therapeutic strategy for neurodegenerative diseases, and activating VCP protein can improve the clearance of such proteins through autophagy and proteasome pathways.

NATURE COMMUNICATIONS (2022)

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Review Cell Biology

Update on the pathogenesis and genetics of Paget's disease of bone

Luigi Gennari et al.

Summary: Studies have revealed major advances in the pathogenesis of Paget's disease of bone (PDB) over the past two decades, particularly in understanding the role of genetic factors. Various germline mutations in different genes have been identified as possible causes of PDB, with most of these genes involved in regulating osteoclast differentiation and function. This review summarizes the most important advances made in the cellular and molecular biology of PDB in recent years.

FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY (2022)

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Review Biochemistry & Molecular Biology

The functional importance of VCP to maintaining cellular protein homeostasis

Brittany A. Ahlstedt et al.

Summary: This review summarizes recent research on VCP, uncovering surprising new ways of regulation, novel aspects of substrate recognition, and novel pathways and substrates that utilize its activity.

BIOCHEMICAL SOCIETY TRANSACTIONS (2022)

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Article Clinical Neurology

Valosin-containing protein Asp395Gly mutation in a patient with frontotemporal dementia: a case report

Ryota Kobayashi et al.

Summary: This study describes the clinical and genetic findings of a patient with pure FTD who carries the p.Asp395Gly VCP variant, which is associated with familial FTD with tauopathy. The patient exhibited self-centered behavior, executive dysfunction, and bilateral frontal lobe atrophy. The absence of comorbid muscle or bone disease suggests that VCP pathogenic variants should be considered in sporadic FTD cases as well. CSF analysis indicated FTD due to NFT accumulation.

BMC NEUROLOGY (2022)

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Article Biochemistry & Molecular Biology

UBXD8 mediates mitochondria-associated degradation to restrain apoptosis and mitophagy

Jing Zheng et al.

Summary: In this study, we reveal the important role of UBXD8 in mitochondrial protein quality control. UBXD8 can recruit VCP to mitochondria and regulate the degradation of substrate proteins in both mitochondria and ER. Additionally, UBXD8 plays a role in apoptosis and mitophagy regulation by degrading certain proteins.

EMBO REPORTS (2022)

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Article Neurosciences

Implications of glial metabolic dysregulation in the pathophysiology of neurodegenerative diseases

Ruqayya Afridi et al.

Summary: Glial cells play a crucial role in maintaining brain homeostasis and defending against inflammation. Metabolic disturbances and chronic neuroinflammation contribute to the activation of glial cells and exacerbate neurodegenerative processes.

NEUROBIOLOGY OF DISEASE (2022)

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Article Cell Biology

Yeast Smy2 and its human homologs GIGYF1 and-2 regulate Cdc48/VCP function during transcription stress

Michelle Harreman Lehner et al.

Summary: The SMY2 gene and its homologs GIGYF1 and GIGYF2 play crucial regulatory roles in the transcription stress response, affecting the function of key factors such as Cdc48/VCP/p97.

CELL REPORTS (2022)

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Article Geriatrics & Gerontology

Global, regional, and national burden of Alzheimer's disease and other dementias, 1990-2019

Xue Li et al.

Summary: From 1990 to 2019, the burden of Alzheimer's disease increased significantly worldwide, with higher increases in high-SDI areas and among the elderly population in those areas. Incidence, prevalence, death, and DALYs rates were positively correlated with the sociodemographic index (SDI), with women having consistently higher rates than men. Risk factors such as smoking for men and obesity for women were identified.

FRONTIERS IN AGING NEUROSCIENCE (2022)

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Review Neurosciences

A survey of protein interactions and posttranslational modifications that influence the polyglutamine diseases

Sean L. Johnson et al.

Summary: The presence and aggregation of misfolded proteins have harmful effects on the nervous system, specifically in the family of polyglutamine (polyQ) disorders. These disorders are caused by the abnormal elongation of a polyQ repeat in different proteins, leading to misfolding, aggregation, cellular dysfunction, and diseases. However, each disease is distinct due to the surrounding regions, interacting proteins, and posttranslational modifications. This overview focuses on the control of pathogenicity in the expanded polyQ repeat by non-polyQ regions and explores shared processes and therapeutic entry points for these incurable disorders.

FRONTIERS IN MOLECULAR NEUROSCIENCE (2022)

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Review Cell Biology

The Primary Cilium and Neuronal Migration

Julie Stoufflet et al.

Summary: The primary cilium plays an important role in regulating cell motility, especially in neuronal migration, by controlling the rhythm and speed of migration.

CELLS (2022)

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Review Cell Biology

Translational regulation by ribosome-associated quality control in neurodegenerative disease, cancer, and viral infection

Bingwei Lu

Summary: Translational control plays a crucial role in protein synthesis, affecting the rate and composition of protein production. It is particularly important for cells under stress and in disease conditions. While much is known about translational control at the initiation step, problems during the elongation and termination steps can lead to ribosome stalling and collision, triggering ribosome-associated quality control mechanisms.

FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY (2022)

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Review Biochemistry & Molecular Biology

Neurodegenerative Microbially-Shaped Diseases: Oxidative Stress Meets Neuroinflammation

Diana Filipa Silva et al.

Summary: Inflammation and oxidative stress play significant roles in neurodegenerative diseases like Alzheimer's and Parkinson's disease. Dysregulation of the immune system, mitochondrial dysfunction, and disrupted gut microbiota composition can lead to chronic systemic inflammation, impair blood-brain barrier function, and promote neuroinflammation and neurodegeneration.

ANTIOXIDANTS (2022)

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Review Biochemistry & Molecular Biology

Amyotrophic Lateral Sclerosis-The Complex Phenotype-From an Epidemiological Perspective: A Focus on Extrapyramidal and Non-Motor Features

Daniele Urso et al.

Summary: This review analyzed available data from epidemiological population-based studies to discuss the extrapyramidal and non-motor features during the course of ALS.

BIOMEDICINES (2022)

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Review Medicine, General & Internal

Coexisting amyotrophic lateral sclerosis and chorea: A case report and literature review

Anni Zhang et al.

Summary: ALS coexisting with chorea is very rare and often hereditary with a genetic mutation, particularly an abnormal amplification of a CAG sequence in the HTT gene. Most patients have a mild course of the disease.

MEDICINE (2022)

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Article Multidisciplinary Sciences

Nanodelivery of nucleic acids

Barbara B. Mendes et al.

Summary: There is a growing need for a safe and effective method to deliver gene therapy materials, and nanomaterials offer an unprecedented opportunity to overcome these challenges. By utilizing their diverse physico-chemical properties, nanomaterials can be functionalized to selectively target specific biomolecules. The combination of gene therapies and nanoscale delivery systems has expanded the therapeutic and biomedical applications of nucleic acid therapeutics.

NATURE REVIEWS METHODS PRIMERS (2022)

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Article Multidisciplinary Sciences

Novel regulation mechanism of adrenal cortisol and DHEA biosynthesis via the endogen ERAD inhibitor small VCP-interacting protein

Recep Ilhan et al.

Summary: This study investigated the regulation mechanism of endoplasmic reticulum-associated degradation (ERAD) and the expression of SVIP (small VCP-interacting protein) in rat adrenal gland. The study revealed that SVIP plays a role in the biosynthesis of cortisol and DHEA, suggesting its potential involvement in the deregulation of steroidogenic stimulator signaling and abnormal adrenal hormone secretion.

SCIENTIFIC REPORTS (2022)

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Review Cell Biology

Organelle-specific autophagy in inflammatory diseases: a potential therapeutic target underlying the quality control of multiple organelles

Ren-Qi Yao et al.

Summary: The structural integrity and functional stability of organelles are crucial for cell viability and responsiveness. Dysfunction of multiple organelles is involved in the pathogenesis and progression of various diseases. Organelle-specific autophagy plays a beneficial role in inflammatory disorders by eliminating damaged organelles and maintaining homeostasis.

AUTOPHAGY (2021)

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Review Cell Biology

Primary cilia biogenesis and associated retinal ciliopathies

Holly Y. Chen et al.

Summary: The primary cilium is a microtubule-based organelle that senses external environment and modulates diverse signaling pathways in different cell types and tissues; ciliopathies are a spectrum of clinical entities caused by aberrant ciliogenesis or ciliary transport, leading to overlapping yet highly variable phenotypes; dysfunction and degeneration of light sensing photoreceptors in the retina are highly penetrant in ciliopathies.

SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY (2021)

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Editorial Material Genetics & Heredity

Ataxia and Parkinsonism in a Woman With a VCP Variant and Long-Normal Repeats in the SCA2 Allele

Alana E. Kirby et al.

Summary: The case presents a 46-year-old woman with gradually progressive ataxia, parkinsonism, and neuropathy. Symptoms began at age 29 and over time, she developed parkinsonism, lower extremity weakness, motor fluctuations, and other complications. She is apparently of European descent and has an unknown family history.

NEUROLOGY-GENETICS (2021)

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Article Chemistry, Multidisciplinary

Gossypol, a novel modulator of VCP, induces autophagic degradation of mutant huntingtin by promoting the formation of VCP/p97-LC3-mHTT complex

Xiao-jing Li et al.

Summary: Huntington's disease is a neurodegenerative disorder caused by toxic aggregates of mutant huntingtin protein in the brain. Inhibiting the activity of Valosin-containing protein, a crucial regulator of proteostasis, could be a potential therapeutic strategy for HD. Gossypol, a drug approved in China, has been identified as a novel modulator of VCP that shows promise in treating HD by reducing levels of mutant huntingtin protein.

ACTA PHARMACOLOGICA SINICA (2021)

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Article Medicine, General & Internal

Spastic Paraplegia with Paget's Disease of Bone due to a VCP Gene Mutation

Takumi Nakamura et al.

Summary: In this case report, a 50-year-old Japanese woman presented with slowly progressing spastic paraplegia and a history of Paget's disease of bone. Genetic testing revealed a mutation of the Valosin-containing protein gene, which has not been previously reported to cause HSP with PDB.

INTERNAL MEDICINE (2021)

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Article Clinical Neurology

An autopsy report of a familial amyotrophic lateral sclerosis case carrying VCP Arg487His mutation with a unique TDP43 proteinopathy

Tomoyasu Matsubara et al.

Summary: This case report presents a unique autopsy case of familial ALS with a VCP gene mutation, showing a predominant involvement of upper motor neurons and later development of frontotemporal dementia symptoms. Pathologically, the patient exhibited features resembling the TDP43 pathology type A pattern.

NEUROPATHOLOGY (2021)

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Article Biochemistry & Molecular Biology

The STRING database in 2021: customizable protein-protein networks, and functional characterization of user-uploaded gene/measurement sets

Damian Szklarczyk et al.

Summary: The STRING database integrates various associations between proteins, including physical interactions and functional associations, by collecting evidence from sources such as literature, databases, computational predictions, and systematic transfers. STRING aims for wide coverage and provides extensive user interface features and querying methods for experimental data.

NUCLEIC ACIDS RESEARCH (2021)

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Article Multidisciplinary Sciences

SVIP is a molecular determinant of lysosomal dynamic stability, neurodegeneration and lifespan

Alyssa E. Johnson et al.

Summary: Valosin-Containing Protein (VCP) is associated with various degenerative diseases. The Small VCP Interacting Protein (SVIP) recruits VCP to lysosomes, with loss or overexpression of SVIP affecting neural degeneration, animal behavior, and lifespan.

NATURE COMMUNICATIONS (2021)

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Article Multidisciplinary Sciences

Seesaw conformations of Npl4 in the human p97 complex and the inhibitory mechanism of a disulfiram derivative

Man Pan et al.

Summary: The human AAA+protein p97 plays a crucial role in cellular protein homeostasis. The study reveals insights into the interaction between p97 and its co-factor Npl4, showing three distinct conformational states of Npl4 in complex with human p97, suggesting the essential role of seesaw motion in the unfolding activity of the p97 complex.

NATURE COMMUNICATIONS (2021)

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Review Cell Biology

Managing risky assets - mitophagy in vivo

Derek P. Narendra

Summary: Mitochondria, resembling their ancestors, are crucial cellular assets producing energy and inducing oxidative stress and inflammation. Mitophagy plays a key role in controlling mitochondrial quality and quantity, with pathways identified in mammals playing important roles in diseases like Parkinson's and Alzheimer's. Despite being essential in disease, mitophagy may not be indispensable under normal physiological conditions, and its roles are being explored in neurodegeneration, cardiomyopathy, metabolism, and red blood cell development.

JOURNAL OF CELL SCIENCE (2021)

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Article Oncology

MEST promotes lung cancer invasion and metastasis by interacting with VCP to activate NF-κB signaling

Yang Wang et al.

Summary: The study identified MEST as a key regulator of invasion and metastasis in lung cancer, promoting cellular metastasis by activating the NF-kappa B signaling pathway. MEST interacts with VCP to coordinate the pathway, and high expressions of MEST and VCP are associated with poor survival in lung cancer patients. Targeting the MEST/VCP/I kappa B alpha/NF-kappa B signaling pathway may be a promising strategy for treating lung cancer.

JOURNAL OF EXPERIMENTAL & CLINICAL CANCER RESEARCH (2021)

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Review Biochemistry & Molecular Biology

Adapting the proteostasis capacity to sustain brain healthspan

Claudio Hetz

Summary: Maintaining neuronal proteostasis is crucial for brain function throughout our lifespan. As we age, the decline in proteostasis network activity may increase the risk of neurodegenerative diseases characterized by abnormal protein aggregates.

CELL (2021)

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Review Medicine, Research & Experimental

Mitochondrial defects: An emerging theranostic avenue towards Alzheimer's associated dysregulations

Shalini Mani et al.

Summary: Mitochondria play a crucial role in energy homeostasis and neuronal cell survival, but dysfunction in mitochondria is associated with neurodegenerative diseases like Alzheimer's. Targeting mitochondria for therapeutic interventions may help delay the onset and progression of Alzheimer's disease.

LIFE SCIENCES (2021)

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Article Biochemistry & Molecular Biology

AAA plus ATPases: structural insertions under the magnifying glass

Matthew Jessop et al.

Summary: AAA+ ATPases are a diverse protein superfamily that drive various cellular processes. Advances in cryo-EM have provided more detailed insights into their structures, leading to a better understanding of their functional relationships and revealing discrepancies in their classification.

CURRENT OPINION IN STRUCTURAL BIOLOGY (2021)

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Article Biochemistry & Molecular Biology

p97 and p47 function in membrane tethering in cooperation with FTCD during mitotic Golgi reassembly

Yayoi Kaneko et al.

Summary: The study identified FTCD as a novel p47-binding protein that plays a role in Golgi reassembly during mitosis by binding to p47 or p97. FTCD, together with p47 and p97, forms a large tethering complex, supporting its role in tethering biological membranes in cooperation with the p97/p47 complex. This suggests that FTCD acts as a tethering factor in p97/p47-mediated Golgi membrane fusion.

EMBO JOURNAL (2021)

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Article Chemistry, Medicinal

A covalent p97/VCP ATPase inhibitor can overcome resistance to CB-5083 and NMS-873 in colorectal cancer cells

Gang Zhang et al.

Summary: Small-molecule inhibitors of p97 are valuable for studying p97 function, and thiol-reactive compounds targeting Cys522 in the D2 ATP-binding domain have shown potential as covalent p97 inhibitors. Proteomic analysis identified PPA as a selective covalent p97 inhibitor with potential for further development to improve potency.

EUROPEAN JOURNAL OF MEDICINAL CHEMISTRY (2021)

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Article Biochemistry & Molecular Biology

VCP/p97 regulates Beclin-1-dependent autophagy initiation

Sandra M. Hill et al.

Summary: VCP is a key regulator in the early initiation of autophagy, playing a role in stabilizing Beclin-1 levels and regulating the activity of the PI3K complex to increase the production of the signaling lipid PI(3)P. Decreased VCP levels or inhibition of its ATPase activity impairs autophagosome formation by limiting downstream recruitment of essential autophagy factors.

NATURE CHEMICAL BIOLOGY (2021)

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Article Biochemistry & Molecular Biology

Reactive astrocytes in ALS display diminished intron retention

Oliver J. Ziff et al.

Summary: This study reveals that decreased intron retention is common in astrocytes carrying ALS-causing mutations, and the overrepresented transcripts involved in reactive transformation are related to processes such as cell adhesion, stress response, and immune activation. The findings suggest a molecular model where poised nuclear reactivity-related IR transcripts are translocated and translated, providing new insights into the molecular regulation of reactive transformation in astrocytes.

NUCLEIC ACIDS RESEARCH (2021)

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Review Clinical Neurology

Genetics of frontotemporal dementia in China

Yaling Jiang et al.

Summary: The study reviewed the genetics of FTD in the Chinese population and found that MAPT is the most common pathogenic gene for FTD in China, with the highest frequency of rare variants in the CHCHD10 gene.

AMYOTROPHIC LATERAL SCLEROSIS AND FRONTOTEMPORAL DEGENERATION (2021)

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Article Biology

Comparative profiling of stress granule clearance reveals differential contributions of the ubiquitin system

Nazife Tolay et al.

Summary: The involvement of the ubiquitin system in stress granule (SG) clearance varies depending on the stress condition, with inhibition of specific components impairing the clearance of SGs induced by certain stressors. This differential involvement is crucial for preventing the formation of disease-related aberrant SGs.

LIFE SCIENCE ALLIANCE (2021)

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Article Cell Biology

USP7 and VCPFAF1 define the SUMO/Ubiquitin landscape at the DNA replication fork

Andre Franz et al.

Summary: The AAA(+)ATPase VCP regulates the extraction of SUMO and ubiquitin-modified DNA replication factors from chromatin, and cooperates with the deubiquitylase USP7 in DNA replication. The role of VCP in chromatin is defined by its cofactor FAF1, which facilitates the extraction of SUMOylated and ubiquitylated proteins. Inactivation of USP7 and FAF1 is synthetically lethal in C. elegans and mammalian cells, supporting a functional link between deubiquitylation and extraction of chromatin-bound proteins.

CELL REPORTS (2021)

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Review Toxicology

The functions and regulation of heat shock proteins; key orchestrators of proteostasis and the heat shock response

Benjamin J. Lang et al.

Summary: Cells respond to protein-damaging stress by activating the Heat Shock Response (HSR), which plays a crucial role in determining cell survival or death. The modulation of HSR and its network have become attractive treatment strategies for neurodegenerative diseases and cancers. A detailed understanding of the mechanisms of HSR is necessary for the development of effective therapies, as important features of HSR continue to be uncovered.

ARCHIVES OF TOXICOLOGY (2021)

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Editorial Material Cell Biology

New functions of a known autophagy regulator: VCP and autophagy initiation

Yuchen Lei et al.

Summary: VCP, a conserved ATPase, regulates autophagy initiation by stabilizing BECN1 and enhancing phosphati-dylinositol 3-kinase (PtdIns3K) complex assembly, in addition to its roles in autophagosome maturation.

AUTOPHAGY (2021)

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Article Genetics & Heredity

A Novel Mutation (D395A) in Valosin-Containing Protein Gene Is Associated With Early Onset Frontotemporal Dementia in an Italian Family

Francesco Bruno et al.

Summary: A new missense heterozygous variant in the VCP gene was identified in three patients from an Italian family, leading to behavioral variant frontotemporal dementia (bvFTD). This highlights the high degree of phenotypic heterogeneity and the importance of genetic screening for VCP mutations in familial bvFTD cases, even in the absence of IBM or PDB signs.

FRONTIERS IN GENETICS (2021)

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Article Virology

Endoplasmic Reticulum-Associated Degradation Controls Virus Protein Homeostasis, Which Is Required for Flavivirus Propagation

Keisuke Tabata et al.

Summary: The study found that the ERAD system controls the amount of each viral protein in virus-infected cells, with viral protein homeostasis critical for viral propagation. The convoluted membrane (CM) serves as a waste dump for viral protein degradation, and the Derlin2/SEL1L/HRD1-specific pathway is involved in this process. This novel ERAD-mediated fine-tuning system for the stoichiometries of polyprotein-derived viral proteins may be a common feature among polyprotein-encoding viruses.

JOURNAL OF VIROLOGY (2021)

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Article Geriatrics & Gerontology

The wide-ranging clinical and genetic features in Japanese families with valosin-containing protein proteinopathy

Takashi Ando et al.

Summary: Mutations in the VCP gene can cause various neurodegenerative disorders, including inclusion body myopathy and amyotrophic lateral sclerosis. This study identified that VCP missense mutations can also lead to demyelinating polyneuropathy, characterized by nerve conduction abnormalities.

NEUROBIOLOGY OF AGING (2021)

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Article Anatomy & Morphology

Valosin-containing Protein in Psoriasis: A Clinical and Immunohistochemical Study

Asmaa Shams El Dein Mohamed et al.

Summary: The study found that VCP expression significantly increased in the skin of patients with psoriasis vulgaris, and was associated with the severity of the lesion and joint involvement. This suggests that VCP may be a useful marker for follow-up and treatment in psoriatic patients.

APPLIED IMMUNOHISTOCHEMISTRY & MOLECULAR MORPHOLOGY (2021)

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Article Cell Biology

Small Molecule Screen Reveals Joint Regulation of Stress Granule Formation and Lipid Droplet Biogenesis

Triana Amen et al.

Summary: Metabolic regulation is essential for stress response pathways, and the interconnection between stress granule formation and lipid droplet accumulation has been uncovered, revealing a novel relationship between stress adaptation mechanisms.

FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY (2021)

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Review Neurosciences

Upper Motor Neuron Disorders: Primary Lateral Sclerosis, Upper Motor Neuron Dominant Amyotrophic Lateral Sclerosis, and Hereditary Spastic Paraplegia

Timothy Fullam et al.

Summary: After ruling out potentially reversible causes, differential diagnosis for patients with a predominant upper motor neuron syndrome includes PLS, HSP, and UMNdALS. Distinguishing between these disorders in the early phases remains challenging, but emerging biomarkers and neuroimaging technologies may be helpful for differentiation. Consensus diagnostic criteria and evolving technologies can improve the precise delineation of PLS from other upper motor neuron disorders and aid in targeted therapeutics for potential disease modification.

BRAIN SCIENCES (2021)

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Review Cell Biology

Cellular functions regulated by deubiquitinating enzymes in neurodegenerative diseases

Hyeon-Ah Do et al.

Summary: Neurodegenerative diseases are common in humans, but their exact pathogenesis is still poorly understood. The ubiquitin-proteasome system (UPS) plays a vital role in protein degradation and maintaining homeostasis, with enzymes like E1, E2, E3 ligases, and deubiquitinating enzymes (DUBs) regulating disease-inducing proteins by controlling ubiquitination. Targeting these enzymes for treatments is emerging as a promising perspective.

AGEING RESEARCH REVIEWS (2021)

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Review Biochemistry & Molecular Biology

DNA damage as a mechanism of neurodegeneration in ALS and a contributor to astrocyte toxicity

Jannigje Rachel Kok et al.

Summary: Increasing evidence supports the involvement of DNA damage in various neurodegenerative diseases, including ALS. DNA damage in ALS may vary between genetic subgroups, particularly with repeat expansion in the C9ORF72 gene. Several genes associated with ALS, such as TARDBP, FUS, NEK1, SQSTM1 and SETX, may contribute to neuronal death by affecting DNA repair and the DNA damage response.

CELLULAR AND MOLECULAR LIFE SCIENCES (2021)

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Article Cell Biology

VCP relocalization limits mitochondrial activity, GSH depletion and ferroptosis during starvation in PC3 prostate cancer cells

Promise Ogor et al.

Summary: During starvation, the VCP protein will relocalize and form aggregate-like structures, leading to decreased mitochondrial activity and cell death. Removal of glutamine triggers VCP relocalization, while its addition to starvation media mitigates this effect.

GENES TO CELLS (2021)

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Review Biochemistry & Molecular Biology

Coordinating DNA Replication and Mitosis through Ubiquitin/SUMO and CDK1

Antonio Galarreta et al.

Summary: Post-translational modification of the DNA replication machinery by ubiquitin and SUMO plays key roles in ensuring the faithful duplication of genetic information. Ubiquitination and SUMOylation not only serve as signals for the extraction of factors from chromatin, but also mediate the disassembly of the replisome and preserve genomic stability in DNA replication.

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2021)

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Review Biochemistry & Molecular Biology

Valosin-Containing Protein (VCP)/p97: A Prognostic Biomarker and Therapeutic Target in Cancer

Susan Costantini et al.

Summary: The review outlines the role of VCP/p97 in different cancers and its potential as a cancer biomarker and therapeutic target, while also addressing the challenges posed by VCP mutations in resistance to inhibitors.

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2021)

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Article Cell Biology

VPS13D interacts with VCP/p97 and negatively regulates endoplasmic reticulum-mitochondria interactions

Yuanjiao Du et al.

Summary: This study demonstrates the key role of VPS13D in negatively regulating ER-mitochondria MCSs, partially through interactions with VCP/p97 to control the level of ER-resident VAPB. Suppression of VPS13D leads to severe defects in mitochondrial morphology, distribution, and DNA synthesis. These results suggest a novel mechanism for the regulation of ER-mitochondria interactions and their impact on cellular functions.

MOLECULAR BIOLOGY OF THE CELL (2021)

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Article Neurosciences

Targeting the VCP-binding motif of ataxin-3 improves phenotypes in Drosophila models of Spinocerebellar Ataxia Type 3

Sean L. Johnson et al.

Summary: SCA3 is the most common polyQ neurodegenerative disease, and targeting the VCP-ataxin-3 interaction has shown potential in reducing ataxin-3 aggregation and toxicity, improving disease phenotypes.

NEUROBIOLOGY OF DISEASE (2021)

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Article Clinical Neurology

Characteristics of VCP mutation-associated cardiomyopathy

Stephani C. Wang et al.

Summary: VCP-associated inclusion body myopathy, Paget's disease of bone, and Frontotemporal Dementia is an autosomal dominant disease caused by mutations in the VCP gene, with patients often experiencing cardiovascular complications in the advanced stages. The study found that affected individuals were older and had a higher prevalence of diastolic dysfunction compared to asymptomatic carriers.

NEUROMUSCULAR DISORDERS (2021)

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Article Multidisciplinary Sciences

Interactome analysis of Bag-1 isoforms reveals novel interaction partners in endoplasmic reticulum-associated degradation

Nisan Denizce Can et al.

Summary: Bag-1 is a multifunctional protein that regulates various cellular processes and has different isoforms with specific interaction networks. The interaction partners of Bag-1 isoforms are involved in protein processing and degradation pathways, suggesting a role for Bag-1 in protein homeostasis. Targeting the interaction surfaces of Bag-1 may provide a potential strategy for cancer treatment.

PLOS ONE (2021)

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Article Multidisciplinary Sciences

Ubiquitination is essential for recovery of cellular activities after heat shock

Brian A. Maxwell et al.

Summary: Stress-induced ubiquitination is crucial for cellular recovery after heat stress, facilitating the degradation of damaged proteins and promoting the resumption of cellular activities.

SCIENCE (2021)

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Article Multidisciplinary Sciences

Ubiquitination of G3BP1 mediates stress granule disassembly in a context-specific manner

Youngdae Gwon et al.

Summary: Stress granules are dynamic condensates composed of RNA and protein that disassemble in a context-dependent manner, with ubiquitination of G3BP1 being required for disassembly in the setting of heat shock.

SCIENCE (2021)

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Article Multidisciplinary Sciences

Nucleolar release of rDNA repeats for repair involves SUMO-mediated untethering by the Cdc48/p97 segregase

Matias Capella et al.

Summary: The authors uncover the mechanism of rDNA release, showing that phosphorylation and SUMOylation facilitate the release of damaged rDNA repeats in the nucleolus. The release of broken rDNA repeats from the nucleolus to the nucleoplasm is essential for repair by homologous recombination and maintaining genome integrity.

NATURE COMMUNICATIONS (2021)

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Article Multidisciplinary Sciences

VCP maintains nuclear size by regulating the DNA damage-associated MDC1-p53-autophagy axis in Drosophila

Ya-Chu Chang et al.

Summary: Disruption of VCP causes progressive nuclear size increase, connecting DNA damage to enlarged nuclei; Accumulation of MDC1 stabilizes p53A, potentially contributing to nuclear size increase; Cells regulate nuclear size by maintaining a constant cytoplasm to nucleus volume ratio, with the role of DNA damage in this process remaining poorly explored.

NATURE COMMUNICATIONS (2021)

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Article Cell Biology

Neuronal VCP loss of function recapitulates FTLD-TDP pathology

Abubakar Wani et al.

Summary: Inactivating VCP and expressing disease-associated mutations in a mouse model revealed that loss of VCP-dependent functions is a mediator of FTLD-TDP, with unexpected biochemical similarity to progranulin deficiency.

CELL REPORTS (2021)

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Review Cell Biology

Current Knowledge of Endolysosomal and Autophagy Defects in Hereditary Spastic Paraplegia

Liriope Toupenet Marchesi et al.

Summary: Hereditary spastic paraplegia (HSP) involves the degeneration of motor neurons and is characterized by clinical and genetic heterogeneity, making it challenging to find effective therapies. Several HSP genes/proteins are linked to the endolysosomal and autophagic pathways, suggesting a functional convergence, which may have implications for understanding the pathological mechanisms of HSP.

CELLS (2021)

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Review Cell Biology

Amyotrophic Lateral Sclerosis (ALS): Stressed by Dysfunctional Mitochondria-Endoplasmic Reticulum Contacts (MERCs)

Junsheng Chen et al.

Summary: ALS is a devastating neurodegenerative disease without cure, with a potential involvement of dysfunctional mitochondria-endoplasmic reticulum contacts (MERCs) leading to altered mitochondrial bioenergetics and oxidative stress. VAPB and VCP are key players in ALS pathogenesis, interacting with other mutant proteins and causing ER stress, inflammation, and motor neuron death. The dysfunction of MERCs in ALS triggers a cascade of pathological changes in the cell.

CELLS (2021)

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Review Cell Biology

Emerging Roles of Ubiquitin-Specific Protease 25 in Diseases

Wenjing Zhu et al.

Summary: The article summarizes the roles of the deubiquitinating enzyme USP25 in disease progression. Previous studies have mainly focused on its role in antiviral immunity and neurodegenerative diseases, but recent research has gradually revealed the mechanisms of action of USP25 in cancer and other diseases.

FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY (2021)

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Review Biochemistry & Molecular Biology

Mitostasis, Calcium and Free Radicals in Health, Aging and Neurodegeneration

Juan A. Godoy et al.

Summary: Mitochondria play critical roles in neurons by regulating neurotransmission and synaptic plasticity through ATP supply, calcium homeostasis, redox balance control, and apoptosis. Their functional integrity is mainly maintained by mitostasis, involving processes like mitochondrial transport, anchoring, fusion, and fission, regulated by various signaling pathways. The communication between mitochondria and the endoplasmic reticulum (ER) via mitochondria-associated membranes (MAMs) is crucial for Ca2+ buffering. Alterations in mitochondrial activity can lead to increased production of reactive oxygen species, disturbance in physiological metabolism, and mitochondrial dysfunction resulting in cell death through apoptosis.

BIOMOLECULES (2021)

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Article Clinical Neurology

TDP-43 and FUS mislocalization in VCP mutant motor neurons is reversed by pharmacological inhibition of the VCP D2 ATPase domain

Jasmine Harley et al.

Summary: RNA binding proteins play a key role in the pathogenesis of ALS, with VCP mutations causing mislocalization of RBPs. The study revealed that enhanced function of the D2 ATPase domain in VCP mutant neurons drives mislocalization of TDP-43 and FUS.

BRAIN COMMUNICATIONS (2021)

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Review Public, Environmental & Occupational Health

Estimated Prevalence and Incidence of Amyotrophic Lateral Sclerosis and SOD1 and C9orf72 Genetic Variants

Carolyn A. Brown et al.

Summary: This study estimated the prevalence and incidence of ALS and SOD1/C9orf72 ALS cases in 22 countries across Europe, North America, Latin America, and Asia. Significant heterogeneity in reported incidence and prevalence was observed within and between countries/geographic regions. The majority of SOD1 and C9orf72 ALS cases were found among those with sporadic ALS, indicating that the classification of familial ALS based on reported family history may not fully capture the genetic origin of ALS.

NEUROEPIDEMIOLOGY (2021)

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Article Cell Biology

p97/VCP inhibition causes excessive MRE11-dependent DNA end resection promoting cell killing after ionizing radiation

Susan Kilgas et al.

Summary: The ATPase p97 interacts with the MRN complex on chromatin and inactivation of p97 affects DNA repair and radiosensitivity. Inhibition of p97 function increases tumor cell killing following ionizing radiation by promoting MRE11 nuclease accumulation, suggesting potential use of p97 inhibitors in radiotherapy patients.

CELL REPORTS (2021)

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Review Clinical Neurology

Multisystem proteinopathy: Where myopathy and motor neuron disease converge

Manisha K. Korb et al.

Summary: Multisystem proteinopathy (MSP) is a group of inherited disorders that cause neurodegeneration, myopathy, and bone disease, sharing common pathophysiology. In addition to mutations in the VCP gene, several other genes are responsible for similar clinical and pathological phenotypes affecting muscle, brain, nerve, and bone.

MUSCLE & NERVE (2021)

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Article Cell Biology

The Autophagy Lysosomal Pathway and Neurodegeneration

Steven Finkbeiner

COLD SPRING HARBOR PERSPECTIVES IN BIOLOGY (2020)

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Article Cell Biology

Cell-Nonautonornous Regulation of Proteostasis in Aging and Disease

Richard I. Morimoto

COLD SPRING HARBOR PERSPECTIVES IN BIOLOGY (2020)

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Article Infectious Diseases

Association of a single nucleotide polymorphism in the ubxn6 gene with long-term non-progression phenotype in HIV-positive individuals

F. Diez-Fuertes et al.

CLINICAL MICROBIOLOGY AND INFECTION (2020)

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Review Biochemistry & Molecular Biology

Repair or Lysophagy: Dealing with Damaged Lysosomes

Chrisovalantis Papadopoulos et al.

JOURNAL OF MOLECULAR BIOLOGY (2020)

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Review Clinical Neurology

Parkinson disease

R. Balestrino et al.

EUROPEAN JOURNAL OF NEUROLOGY (2020)

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Article Chemistry, Medicinal

p97: An Emerging Target for Cancer, Neurodegenerative Diseases, and Viral Infections

Donna M. Huryn et al.

JOURNAL OF MEDICINAL CHEMISTRY (2020)

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Article Biochemistry & Molecular Biology

ClinVar: improvements to accessing data

Melissa J. Landrum et al.

NUCLEIC ACIDS RESEARCH (2020)

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Article Biochemistry & Molecular Biology

VCP/p97 targets the nuclear export and degradation of p27Kip1 during G1 to S phase transition

Xianli Shi et al.

FASEB JOURNAL (2020)

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Article Biotechnology & Applied Microbiology

Treatment of a Mouse Model of ALS by In Vivo Base Editing

Colin K. W. Lim et al.

MOLECULAR THERAPY (2020)

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Article Clinical Neurology

Phenotypic convergence in Charcot-Marie-Tooth 2Y with novel VCP mutation

Jasmine Gite et al.

NEUROMUSCULAR DISORDERS (2020)

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Article Multidisciplinary Sciences

Safety and effectiveness of a novel neuroprotectant, KUS121, in patients with non-arteritic central retinal artery occlusion: An open-label, non-randomized, first-in-humans, phase 1/2 trial

Hanako Ohashi Ikeda et al.

PLOS ONE (2020)

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Article Multidisciplinary Sciences

Golgi organization is regulated by proteasomal degradation

Avital Eisenberg-Lerner et al.

NATURE COMMUNICATIONS (2020)

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Article Medicine, General & Internal

Sorting Rare ALS Genetic Variants by Targeted Re-Sequencing Panel in Italian Patients: OPTN, VCP, and SQSTM1 Variants Account for 3% of Rare Genetic Forms

Viviana Pensato et al.

JOURNAL OF CLINICAL MEDICINE (2020)

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Article Multidisciplinary Sciences

VCP expression decrease as a biomarker of preclinical and early clinical stages of Parkinson's disease

Anelya Alieva et al.

SCIENTIFIC REPORTS (2020)

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Review Biochemistry & Molecular Biology

Valosin-Containing Protein, a Calcium-Associated ATPase Protein, in Endoplasmic Reticulum and Mitochondrial Function and Its Implications for Diseases

Xiaonan Sun et al.

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2020)

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Article Multidisciplinary Sciences

TEX264 coordinates p97-and SPRTN-mediated resolution of topoisomerase 1-DNA adducts

John Fielden et al.

NATURE COMMUNICATIONS (2020)

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Article Cell Biology

Drosophila VCP/p97 Mediates Dynein-Dependent Retrograde Mitochondrial Motility in Axons

Ashley E. Gonzalez et al.

FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY (2020)

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Article Cell Biology

Phospho-Ser(784)-VCP Is Required for DNA Damage Response and Is Associated with Poor Prognosis of Chemotherapy-Treated Breast Cancer

Cuige Zhu et al.

CELL REPORTS (2020)

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Article Cell Biology

Vcp Overexpression and Leucine Supplementation Increase Protein Synthesis and Improve Fear Memory and Social Interaction of Nf1 Mutant Mice

Yu-Tzu Shih et al.

CELL REPORTS (2020)

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Article Cell Biology

The Cdc48 Complex Alleviates the Cytotoxicity of Misfolded Proteins by Regulating Ubiquitin Homeostasis

Ryan Higgins et al.

CELL REPORTS (2020)

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Review Biochemistry & Molecular Biology

Mitochondrial Surveillance by Cdc48/p97: MAD vs. Membrane Fusion

Mafalda Escobar-Henriques et al.

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2020)

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Article Neurosciences

The Integrated UPR and ERAD in Oligodendrocytes Maintain Myelin Thickness in Adults by Regulating Myelin Protein Translation

Shuangchan Wu et al.

JOURNAL OF NEUROSCIENCE (2020)

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Article Multidisciplinary Sciences

Quality-control mechanisms targeting translationally stalled and C-terminally extended poly(GR) associated with ALS/FTD

Shuangxi Li et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2020)

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Article Multidisciplinary Sciences

Autosomal dominant VCP hypomorph mutation impairs disaggregation of PHF-tau

Nabil F. Darwich et al.

SCIENCE (2020)

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Article Oncology

Systems Biology Approaches Reveal Potential Phenotype-Modifier Genes in Neurofibromatosis Type 1

Thayne Woycinck Kowalski et al.

CANCERS (2020)

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Letter Clinical Neurology

FUS is lost from nuclei and gained in neurites of motor neurons in a human stem cell model of VCP-related ALS

Jasmine Harley et al.

BRAIN (2020)

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Article Biochemistry & Molecular Biology

Ceramide contributes to pathogenesis and may be targeted for therapy in VCP inclusion body myopathy

Lan Weiss et al.

Summary: Manipulating ceramide levels in myoblast cultures from VCPR155H mutant mice and VCP patients demonstrated the potential role of ceramide in VCP disease. The ceramide pathway appears critical in the pathogenesis of VCP, and small-molecule inhibitors of ceramide biosynthesis may provide therapeutic benefits in VCP and related neurodegenerative diseases.

HUMAN MOLECULAR GENETICS (2020)

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Review Neurosciences

Cellular Proteostasis in Neurodegeneration

Alberim Kurtishi et al.

MOLECULAR NEUROBIOLOGY (2019)

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Review Cell Biology

The proteostasis network and its decline in ageing

Mark S. Hipp et al.

NATURE REVIEWS MOLECULAR CELL BIOLOGY (2019)

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Article Genetics & Heredity

Is the High Frequency of Machado-Joseph Disease in China Due to New Mutational Origins?

Tianjiao Li et al.

FRONTIERS IN GENETICS (2019)

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Article Biochemistry & Molecular Biology

ULK1 and ULK2 Regulate Stress Granule Disassembly Through Phosphorylation and Activation of VCP/p97

Bo Wang et al.

MOLECULAR CELL (2019)

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Review Cell Biology

Mechanisms and functions of ribosome-associated protein quality control

Claudio A. P. Joazeiro

NATURE REVIEWS MOLECULAR CELL BIOLOGY (2019)

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Review Biochemistry & Molecular Biology

Modulating protein-protein interaction networks in protein homeostasis

Mengqi Zhong et al.

CURRENT OPINION IN CHEMICAL BIOLOGY (2019)

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Review Genetics & Heredity

Proteolytic control of genome integrity at the replication fork

Julie Rageul et al.

DNA REPAIR (2019)

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Article Psychiatry

Mutation and association analyses of dementia-causal genes in Han Chinese patients with early-onset and familial Alzheimer's disease

Guihong Wang et al.

JOURNAL OF PSYCHIATRIC RESEARCH (2019)

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Article Multidisciplinary Sciences

Structure of the Cdc48 segregase in the act of unfolding an authentic substrate

Ian Cooney et al.

SCIENCE (2019)

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Article Multidisciplinary Sciences

Substrate processing by the Cdc48 ATPase complex is initiated by ubiquitin unfolding

Edward C. Twomey et al.

SCIENCE (2019)

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Article Cell Biology

VCP Machinery Mediates Autophagic Degradation of Empty Argonaute

Hotaka Kobayashi et al.

CELL REPORTS (2019)

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Review Cell Biology

The Mitochondria-Endoplasmic Reticulum Contacts and Their Critical Role in Aging and Age-Associated Diseases

Ornella Moltedo et al.

FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY (2019)

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Article Biochemistry & Molecular Biology

The p97-Ataxin 3 complex regulates homeostasis of the DNA damage response E3 ubiquitin ligase RNF8

Abhay Narayan Singh et al.

EMBO JOURNAL (2019)

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Article Biochemistry & Molecular Biology

Parkin-mediated ubiquitylation redistributes MITOL/March5 from mitochondria to peroxisomes

Fumika Koyano et al.

EMBO REPORTS (2019)

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Review Biochemistry & Molecular Biology

Properties of Stress Granule and P-Body Proteomes

Ji-Young Youn et al.

MOLECULAR CELL (2019)

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Article Pharmacology & Pharmacy

Differences between acute and chronic stress granules, and how these differences may impact function in human disease

Lucas C. Reineke et al.

BIOCHEMICAL PHARMACOLOGY (2019)

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Article Multidisciplinary Sciences

Integrated proteogenetic analysis reveals the landscape of a mitochondrial-autophagosome synapse during PARK2-dependent mitophagy

Jin-Mi Heo et al.

SCIENCE ADVANCES (2019)

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Article Biochemical Research Methods

VarSome: the human genomic variant search engine

Christos Kopanos et al.

BIOINFORMATICS (2019)

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Article Biochemistry & Molecular Biology

Physiological and pathophysiological characteristics of ataxin-3 isoforms

Daniel Weishaeupl et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2019)

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Article Geriatrics & Gerontology

Frontotemporal dementia spectrum: first genetic screen in a Greek cohort

Eliana Marisa Ramos et al.

NEUROBIOLOGY OF AGING (2019)

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Article Biochemistry & Molecular Biology

Multisystem Proteinopathy Mutations in VCP/p97 Increase NPLOC4.UFD1L Binding and Substrate Processing

Emily E. Blythe et al.

STRUCTURE (2019)

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Review Clinical Neurology

Huntington's disease: a clinical review

P. McColgan et al.

EUROPEAN JOURNAL OF NEUROLOGY (2018)

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Review Cell Biology

Pathways of cellular proteostasis in aging and disease

Courtney L. Klaips et al.

JOURNAL OF CELL BIOLOGY (2018)

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Review Biochemistry & Molecular Biology

Protein Quality Control of the Endoplasmic Reticulum and Ubiquitin-Proteasome-Triggered Degradation of Aberrant Proteins: Yeast Pioneers the Path

Nicole Berner et al.

ANNUAL REVIEW OF BIOCHEMISTRY, VOL 87 (2018)

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Article Cell Biology

Loss of the novel Vcp (valosin containing protein) interactor Washc4 interferes with autophagy-mediated proteostasis in striated muscle and leads to myopathy in vivo

Monika Kustermann et al.

AUTOPHAGY (2018)

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Article Oncology

PTPN12/PTP-PEST Regulates Phosphorylation-Dependent Ubiquitination and Stability of Focal Adhesion Substrates in Invasive Glioblastoma Cells

Zhihua Chen et al.

CANCER RESEARCH (2018)

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Article Geriatrics & Gerontology

Cytoplasmic RNA Granules in Somatic Maintenance

Ossama Moujaber et al.

GERONTOLOGY (2018)

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Article Genetics & Heredity

ClinVar Miner: Demonstrating utility of a Web-based tool for viewing and filtering ClinVar data

Alex Henrie et al.

HUMAN MUTATION (2018)

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Article Biochemistry & Molecular Biology

Interaction between the AAA ATPase p97/VCP and a concealed UBX domain in the copper transporter ATP7A is associated with motor neuron degeneration

Ling Yi et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2018)

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Article Biochemical Research Methods

AP-SWATH Reveals Direct Involvement of VCP/p97 in Integrated Stress Response Signaling Through Facilitating CReP/PPP1R15B Degradation

Julia Huelsmann et al.

MOLECULAR & CELLULAR PROTEOMICS (2018)

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Review Biochemistry & Molecular Biology

VCP/p97-Mediated Unfolding as a Principle in Protein Homeostasis and Signaling

Johannes van den Boom et al.

MOLECULAR CELL (2018)

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Article Biochemistry & Molecular Biology

ZFAND1 Recruits p97 and the 26S Proteasome to Promote the Clearance of Arsenite-Induced Stress Granules

Ankit Turakhiya et al.

MOLECULAR CELL (2018)

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Review Clinical Neurology

Tau-targeting therapies for Alzheimer disease

Erin E. Congdon et al.

NATURE REVIEWS NEUROLOGY (2018)

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Article Geriatrics & Gerontology

Molecular epidemiological study of familial amyotrophic lateral sclerosis in Japanese population by whole-exome sequencing and identification of novel HNRNPA1 mutation

Hiroya Naruse et al.

NEUROBIOLOGY OF AGING (2018)

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Article Geriatrics & Gerontology

Investigating CCNF mutations in a Taiwanese cohort with amyotrophic lateral sclerosis

Pei-Chien Tsai et al.

NEUROBIOLOGY OF AGING (2018)

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Article Neurosciences

Toxicity and aggregation of the polyglutamine disease protein, ataxin-3 is regulated by its binding to VCP/p97 in Drosophila melanogaster

Gorica Ristic et al.

NEUROBIOLOGY OF DISEASE (2018)

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Article Multidisciplinary Sciences

Hsp70-Bag3 complex is a hub for proteotoxicity-induced signaling that controls protein aggregation

Anatoli B. Meriin et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2018)

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Review Biochemistry & Molecular Biology

Quality Control in the Endoplasmic Reticulum: Crosstalk between ERAD and UPR pathways

Jiwon Hwang et al.

TRENDS IN BIOCHEMICAL SCIENCES (2018)

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Article Biochemistry & Molecular Biology

The heterozygous R155C VCP mutation: Toxic in humans! Harmless in mice?

Christoph S. Clemen et al.

BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS (2018)

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Article Biochemistry & Molecular Biology

The ATPase VCP/p97 functions as a disaggregase against toxic Huntingtin-exonl aggregates

Debasish Kumar Ghosh et al.

FEBS LETTERS (2018)

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Article Multidisciplinary Sciences

Vmsl and ANKZF1 peptidyl-tRNA hydrolases release nascent chains from stalled ribosomes

Rati Verma et al.

NATURE (2018)

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Article Pharmacology & Pharmacy

Neuronal and astrocytic primary cilia in the mature brain

Ashley Sterpka et al.

PHARMACOLOGICAL RESEARCH (2018)

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Review Cell Biology

The involvement of endoplasmic reticulum formation and protein synthesis efficiency in VCP- and ATL1-related neurological disorders

Yu-Tzu Shih et al.

JOURNAL OF BIOMEDICAL SCIENCE (2018)

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Article Genetics & Heredity

Genotype-phenotype study in patients with valosin-containing protein mutations associated with multisystem proteinopathy

E. Al-Obeidi et al.

CLINICAL GENETICS (2018)

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Article Biochemistry & Molecular Biology

VCP/p97 cooperates with YOD1, UBXD1 and PLAA to drive clearance of ruptured lysosomes by autophagy

Chrisovalantis Papadopoulos et al.

EMBO JOURNAL (2017)

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Article Cell Biology

The opposite role of two UBA-UBX containing proteins, p47 and SAKS1 in the degradation of a single ERAD substrate, α-TCR

Eun Sil Park et al.

MOLECULAR AND CELLULAR BIOCHEMISTRY (2017)

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Review Biochemistry & Molecular Biology

Repair or destruction-an intimate liaison between ubiquitin ligases and molecular chaperones in proteostasis

Eva Kevei et al.

FEBS LETTERS (2017)

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Article Biochemistry & Molecular Biology

Mutations in valosin-containing protein (VCP) decrease ADP/ATP translocation across the mitochondrial membrane and impair energy metabolism in human neurons

Marthe H. R. Ludtmann et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2017)

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Letter Clinical Neurology

Parkinsonism in a patient with valosin-containing protein gene mutation showing: a case report

Motoki Fujimaki et al.

JOURNAL OF NEUROLOGY (2017)

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Review Cell Biology

Modulation of valosin-containing protein by Kyoto University Substances (KUS) as a novel therapeutic strategy for ischemic neuronal diseases

Masayuki Hata et al.

NEURAL REGENERATION RESEARCH (2017)

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Editorial Material Clinical Neurology

VCP-RELATED MULTISYSTEM PROTEINOPATHY PRESENTING AS EARLY-ONSET PARKINSON DISEASE

Martin Regensburger et al.

NEUROLOGY (2017)

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Article Neurosciences

TIA1 Mutations in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia Promote Phase Separation and Alter Stress Granule Dynamics

Ian R. Mackenzie et al.

NEURON (2017)

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Article Multidisciplinary Sciences

Cytosolic Fc receptor TRIM21 inhibits seeded tau aggregation

William A. McEwan et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2017)

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Review Neurosciences

Optineurin in amyotrophic lateral sclerosis: Multifunctional adaptor protein at the crossroads of different neuroprotective mechanisms

Andrea Markovinovic et al.

PROGRESS IN NEUROBIOLOGY (2017)

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Article Cell Biology

Progressive Motor Neuron Pathology and the Role of Astrocytes in a Human Stem Cell Model of VCP-Related ALS

Claire E. Hall et al.

CELL REPORTS (2017)

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Article Biology

Valosin-containing protein (VCP/p97) inhibitors relieve Mitofusin-dependent mitochondrial defects due to VCP disease mutants

Ting Zhang et al.

ELIFE (2017)

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Review Neurosciences

Granulostasis: Protein Quality Control of RNP Granules

Simon Alberti et al.

FRONTIERS IN MOLECULAR NEUROSCIENCE (2017)

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Article Cell Biology

Aurora-A promotes the establishment of spindle assembly checkpoint by priming the Haspin-Aurora-B feedback loop in late G2 phase

Fazhi Yu et al.

CELL DISCOVERY (2017)

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Article Multidisciplinary Sciences

The spliceosome-associated protein Mfap1 binds to VCP in Drosophila

Sandra Rode et al.

PLOS ONE (2017)

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Review Biochemistry & Molecular Biology

Detection and Clearance of Damaged Lysosomes by the Endo-Lysosomal Damage Response and Lysophagy

Chrisovalantis Papadopoulos et al.

CURRENT BIOLOGY (2017)

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Article Biochemical Research Methods

ProtVista: visualization of protein sequence annotations

Xavier Watkins et al.

BIOINFORMATICS (2017)

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Article Clinical Neurology

Proteomic differences in amyloid plaques in rapidly progressive and sporadic Alzheimer's disease

Eleanor Drummond et al.

ACTA NEUROPATHOLOGICA (2017)

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Article Biochemistry & Molecular Biology

Molecular Mechanism of Substrate Processing by the Cdc48 ATPase Complex

Nicholas O. Bodnar et al.

CELL (2017)

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Article Genetics & Heredity

PLAA Mutations Cause a Lethal Infantile Epileptic Encephalopathy by Disrupting Ubiquitin-Mediated Endolysosomal Degradation of Synaptic Proteins

Emma A. Hall et al.

AMERICAN JOURNAL OF HUMAN GENETICS (2017)

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Article Biochemistry & Molecular Biology

Selenoprotein S is required for clearance of C99 through endoplasmic reticulum-associated degradation

Jun Ki Jang et al.

BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS (2017)

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Review Biochemistry & Molecular Biology

Cytoplasmic stress granules: Dynamic modulators of cell signaling and disease

Hicham Mahboubi et al.

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE (2017)

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Article Biochemistry & Molecular Biology

VCP cooperates with UBXD1 to degrade mitochondrial outer membrane protein MCL1 in model of Huntington's disease

Xing Guo et al.

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE (2017)

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Review Biochemistry & Molecular Biology

The Interplay of Cofactor Interactions and Post-translational Modifications in the Regulation of the AAA+ ATPase p97

Petra Haenzelmann et al.

FRONTIERS IN MOLECULAR BIOSCIENCES (2017)

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Article Pathology

The Myoblast C2C12 Transfected with Mutant Valosin-Containing Protein Exhibits Delayed Stress Granule Resolution on Oxidative Stress

Carlos J. Rodriguez-Ortiz et al.

AMERICAN JOURNAL OF PATHOLOGY (2016)

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Review Cell Biology

The life cycle of the 26S proteasome: from birth, through regulation and function, and onto its death

Ido Livneh et al.

CELL RESEARCH (2016)

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Article Cell Biology

Monoubiquitination of Syntaxin 5 Regulates Golgi Membrane Dynamics during the Cell Cycle

Shijiao Huang et al.

DEVELOPMENTAL CELL (2016)

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Article Biochemistry & Molecular Biology

Clinical exome sequencing for cerebellar ataxia and spastic paraplegia uncovers novel gene-disease associations and unanticipated rare disorders

Bart P. van De Warrenburg et al.

EUROPEAN JOURNAL OF HUMAN GENETICS (2016)

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Article Biochemistry & Molecular Biology

Silencing of Small Valosin-containing Protein-interacting Protein (SVIP) Reduces Very Low Density Lipoprotein (VLDL) Secretion from Rat Hepatocytes by Disrupting Its Endoplasmic Reticulum (ER)-to-Golgi Trafficking

Samata Tiwari et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2016)

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Article Biochemistry & Molecular Biology

Pathogenic Mutations in the Valosin-containing Protein/p97(VCP) N-domain Inhibit the SUMOylation of VCP and Lead to Impaired Stress Response

Tao Wang et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2016)

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Article Biochemistry & Molecular Biology

The Ankrd13 Family of Ubiquitin-interacting Motif-bearing Proteins Regulates Valosin-containing Protein/p97 Protein-mediated Lysosomal Trafficking of Caveolin 1

Daocharad Burana et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2016)

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Article Cell Biology

Doa1 targets ubiquitinated substrates for mitochondria-associated degradation

Xi Wu et al.

JOURNAL OF CELL BIOLOGY (2016)

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Review Biochemistry & Molecular Biology

Origin and Functional Evolution of the Cdc48/p97/VCP AAA+ Protein Unfolding and Remodeling Machine

Dominik Barthelme et al.

JOURNAL OF MOLECULAR BIOLOGY (2016)

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Article Biochemical Research Methods

Identification of Palmitoylated Transitional Endoplasmic Reticulum ATPase by Proteomic Technique and Pan Antipalmitoylation Antibody

Caiyun Fang et al.

JOURNAL OF PROTEOME RESEARCH (2016)

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Review Clinical Neurology

Heat shock proteins as potential targets for protective strategies in neurodegeneration

Harm H. Kampinga et al.

LANCET NEUROLOGY (2016)

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Article Biochemical Research Methods

Valosin-containing protein (VCP)-Adaptor Interactions are Exceptionally Dynamic and Subject to Differential Modulation by a VCP Inhibitor

Liang Xue et al.

MOLECULAR & CELLULAR PROTEOMICS (2016)

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Article Multidisciplinary Sciences

Failure of RQC machinery causes protein aggregation and proteotoxic stress

Young-Jun Choe et al.

NATURE (2016)

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Review Clinical Neurology

Parkinsonism, movement disorders and genetics in frontotemporal dementia

Jose Fidel Baizabal-Carvallo et al.

NATURE REVIEWS NEUROLOGY (2016)

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Article Geriatrics & Gerontology

Mutational analysis of TBK1 in Taiwanese patients with amyotrophic lateral sclerosis

Pei-Chien Tsai et al.

NEUROBIOLOGY OF AGING (2016)

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Article Geriatrics & Gerontology

Rare variants in SQSTM1 and VCP genes and risk of sporadic inclusion body myositis

Qiang Gang et al.

NEUROBIOLOGY OF AGING (2016)

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Article Multidisciplinary Sciences

IBMPFD Disease-Causing Mutant VCP/p97 Proteins Are Targets of Autophagic-Lysosomal Degradation

Oznur Bayraktar et al.

PLOS ONE (2016)

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Article Cell Biology

Targeting protein homeostasis in sporadic inclusion body myositis

Mhoriam Ahmed et al.

SCIENCE TRANSLATIONAL MEDICINE (2016)

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Article Biochemistry & Molecular Biology

Structural Basis of ATP Hydrolysis and Intersubunit Signaling in the AAA+ ATPase p97

Petra Haenzelmann et al.

STRUCTURE (2016)

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Review Immunology

Cellular Functions of Optineurin in Health and Disease

Karolina Slowicka et al.

TRENDS IN IMMUNOLOGY (2016)

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Article Multidisciplinary Sciences

VCP and ATL1 regulate endoplasmic reticulum and protein synthesis for dendritic spine formation

Yu-Tzu Shih et al.

NATURE COMMUNICATIONS (2016)

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Article Multidisciplinary Sciences

VCP recruitment to mitochondria causes mitophagy impairment and neurodegeneration in models of Huntington's disease

Xing Guo et al.

NATURE COMMUNICATIONS (2016)

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Article Mathematical & Computational Biology

Genic insights from integrated human proteomics in GeneCards

Simon Fishilevich et al.

DATABASE-THE JOURNAL OF BIOLOGICAL DATABASES AND CURATION (2016)

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Article Clinical Neurology

Mutations in FUS are the most frequent genetic cause in juvenile sporadic ALS patients of Chinese origin

Zhang-Yu Zou et al.

AMYOTROPHIC LATERAL SCLEROSIS AND FRONTOTEMPORAL DEGENERATION (2016)

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Review Neurosciences

The Involvement of Neuron-Specific Factors in Dendritic Spinogenesis: Molecular Regulation and Association with Neurological Disorders

Hsiao-Tang Hu et al.

NEURAL PLASTICITY (2016)

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Review Biochemistry & Molecular Biology

The Biology of Proteostasis in Aging and Disease

Johnathan Labbadia et al.

ANNUAL REVIEW OF BIOCHEMISTRY, VOL 84 (2015)

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Article Biochemistry & Molecular Biology

Nucleocytoplasmic shuttling of valosin-containing protein (VCP/p97) regulated by its N domain and C-terminal region

Changcheng Song et al.

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH (2015)

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Article Oncology

Targeting the AAA ATPase p97 as an Approach to Treat Cancer through Disruption of Protein Homeostasis

Daniel J. Anderson et al.

CANCER CELL (2015)

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Article Biochemistry & Molecular Biology

A non-canonical role of the p97 complex in RIG-I antiviral signaling

Qian Hao et al.

EMBO JOURNAL (2015)

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Article Biochemistry & Molecular Biology

The VCP/p97 and YOD1 Proteins Have Different Substrate-dependent Activities in Endoplasmic Reticulum-associated Degradation (ERAD)

Linda Sasset et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2015)

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Article Biochemistry & Molecular Biology

Selenoprotein S-dependent Selenoprotein K Binding to p97(VCP) Protein Is Essential for Endoplasmic Reticulum-associated Degradation

Jea Hwang Lee et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2015)

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Article Cell Biology

Systematic proteomics of the VCP-UBXD adaptor network identifies a role for UBXN10 in regulating ciliogenesis

Malavika Raman et al.

NATURE CELL BIOLOGY (2015)

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Article Geriatrics & Gerontology

VCP gene analyses in Japanese patients with sporadic amyotrophic lateral sclerosis identify a new mutation

Makito Hirano et al.

NEUROBIOLOGY OF AGING (2015)

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Article Geriatrics & Gerontology

Mutational analysis of MATR3 in Taiwanese patients with amyotrophic lateral sclerosis

Kon-Ping Lin et al.

NEUROBIOLOGY OF AGING (2015)

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Editorial Material Clinical Neurology

Multisystem proteinopathy Intersecting genetics in muscle, bone, and brain degeneration

J. Paul Taylor

NEUROLOGY (2015)

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Altered cofactor regulation with disease-associated p97/VCP mutations

Xiaoyi Zhang et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2015)

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Article Biochemistry & Molecular Biology

An ALS disease mutation in Cdc48/p97 impairs 20S proteasome binding and proteolytic communication

Dominik Barthelme et al.

PROTEIN SCIENCE (2015)

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Wss1 metalloprotease partners with Cdc48/Doa1 in processing genotoxic SUMO conjugates

Maxim Y Balakirev et al.

eLife (2015)

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Review Genetics & Heredity

Neurogenetic Disorders in the Basque Population

Jose Felix Marti Masso et al.

ANNALS OF HUMAN GENETICS (2015)

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PhosphoSitePlus, 2014: mutations, PTMs and recalibrations

Peter V. Hornbeck et al.

NUCLEIC ACIDS RESEARCH (2015)

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The BioGRID interaction database: 2015 update

Andrew Chatr-aryamontri et al.

NUCLEIC ACIDS RESEARCH (2015)

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A novel mutation in VCP causes Charcot-Marie-Tooth Type 2 disease

Michael A. Gonzalez et al.

BRAIN (2014)

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Inhibition of autophagy, lysosome and VCP function impairs stress granule assembly

S. J. Seguin et al.

CELL DEATH AND DIFFERENTIATION (2014)

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Lipid-enriched diet rescues lethality and slows down progression in a murine model of VCP-associated disease

Katrina J. Llewellyn et al.

HUMAN MOLECULAR GENETICS (2014)

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Article Biochemistry & Molecular Biology

Identification of ter94, Drosophila VCP, as a strong modulator of motor neuron degeneration induced by knockdown of Caz, Drosophila FUS

Yumiko Azuma et al.

HUMAN MOLECULAR GENETICS (2014)

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Article Biochemistry & Molecular Biology

Sequential Actions of the AAA-ATPase Valosin-containing Protein (VCP)/p97 and the Proteasome 19 S Regulatory Particle in Sterol-accelerated, Endoplasmic Reticulum (ER)associated Degradation of 3-Hydroxy-3-methylglutaryl-coenzyme A Reductase

Lindsey L. Morris et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2014)

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Article Biochemistry & Molecular Biology

Pathogenic mutation of UBQLN2 impairs its interaction with UBXD8 and disrupts endoplasmic reticulum-associated protein degradation

Yuxing Xia et al.

JOURNAL OF NEUROCHEMISTRY (2014)

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Binding of OTULIN to the PUB Domain of HOIP Controls NF-κB Signaling

Veronique Schaeffer et al.

MOLECULAR CELL (2014)

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Article Geriatrics & Gerontology

Extensive molecular genetic survey of Taiwanese patients with amyotrophic lateral sclerosis

Bing-Wen Soong et al.

NEUROBIOLOGY OF AGING (2014)

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Review Neurosciences

Autophagy and apoptosis dysfunction in neurodegenerative disorders

Saeid Ghavami et al.

PROGRESS IN NEUROBIOLOGY (2014)

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The Reference Genome Sequence of Saccharomyces cerevisiae: Then and Now

Stacia R. Engel et al.

G3-GENES GENOMES GENETICS (2014)

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Derlin-1 Regulates Mutant VCP-Linked Pathogenesis and Endoplasmic Reticulum Stress-Induced Apoptosis

Cyong-Jhih Liang et al.

PLOS GENETICS (2014)

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Immunoreactivity of valosin-containing protein in sporadic amyotrophic lateral sclerosis and in a case of its novel mutant

Takashi Ayaki et al.

ACTA NEUROPATHOLOGICA COMMUNICATIONS (2014)

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Signal-peptide-mediated translocation is regulated by a p97–AIRAPL complex

Tal Glinka et al.

BIOCHEMICAL JOURNAL (2013)

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Eukaryotic Stress Granules Are Cleared by Autophagy and Cdc48/VCP Function

J. Ross Buchan et al.

CELL (2013)

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A unique IBMPFD-related P97/VCP mutation with differential binding pattern and subcellular localization

Yalcin Erzurumlu et al.

INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY (2013)

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Article Biochemistry & Molecular Biology

Altered Intersubunit Communication Is the Molecular Basis for Functional Defects of Pathogenic p97 Mutants

Wai Kwan Tang et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2013)

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Article Biochemistry & Molecular Biology

Interaction between Salt-inducible Kinase 2 (SIK2) and p97/Valosin-containing Protein (VCP) Regulates Endoplasmic Reticulum (ER)-associated Protein Degradation in Mammalian Cells

Fu-Chia Yang et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2013)

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Article Cell Biology

The UBXN-2/p37/p47 adaptors of CDC-48/p97 regulate mitosis by limiting the centrosomal recruitment of Aurora A

Elsa Kress et al.

JOURNAL OF CELL BIOLOGY (2013)

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Characterization of the Drosophila Atlastin Interactome Reveals VCP as a Functionally Related Interactor

Niamh C. O'Sullivan et al.

JOURNAL OF GENETICS AND GENOMICS (2013)

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Delineating the genetic heterogeneity of ALS using targeted high-throughput sequencing

Kevin P. Kenna et al.

JOURNAL OF MEDICAL GENETICS (2013)

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Covalent and allosteric inhibitors of the ATPase VCP/p97 induce cancer cell death

Paola Magnaghi et al.

NATURE CHEMICAL BIOLOGY (2013)

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Novel valosin containing protein mutation in a Swiss family with hereditary inclusion body myopathy and dementia

Anne-Kathrin Peyer et al.

NEUROMUSCULAR DISORDERS (2013)

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Pathogenic VCP Mutations Induce Mitochondrial Uncoupling and Reduced ATP Levels

Fernando Bartolome et al.

NEURON (2013)

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VCP Is Essential for Mitochondrial Quality Control by PINK1/Parkin and this Function Is Impaired by VCP Mutations

Nam Chul Kim et al.

NEURON (2013)

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Valosin-containing protein immunoreactivity in tauopathies, synucleinopathies, polyglutamine diseases and intranuclear inclusion body disease

Fumiaki Mori et al.

NEUROPATHOLOGY (2013)

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Spatial regulation of UBXD8 and p97/VCP controls ATGL-mediated lipid droplet turnover

James A. Olzmann et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2013)

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A functional deficiency of TERA/VCP/p97 contributes to impaired DNA repair in multiple polyglutamine diseases

Kyota Fujita et al.

NATURE COMMUNICATIONS (2013)

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Soluble forms of polyQ-expanded huntingtin rather than large aggregates cause endoplasmic reticulum stress

Julia Leitman et al.

NATURE COMMUNICATIONS (2013)

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p97 complexes as signal integration hubs

Hemmo Meyer

BMC BIOLOGY (2012)

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Letter Clinical Neurology

Hereditary spastic paraplegia caused by a mutation in the VCP gene

Susanne T. de Bot et al.

BRAIN (2012)

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A Ribosome-Bound Quality Control Complex Triggers Degradation of Nascent Peptides and Signals Translation Stress

Onn Brandman et al.

CELL (2012)

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Characterization of the Asian myopathy patients with VCP mutations

Z. Shi et al.

EUROPEAN JOURNAL OF NEUROLOGY (2012)

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Clinical, pathological, and genetic mutation analysis of sporadic inclusion body myositis in Japanese people

Huaying Cai et al.

JOURNAL OF NEUROLOGY (2012)

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Mutation analysis of VCP in British familial and sporadic amyotrophic lateral sclerosis patients

Jack W. Miller et al.

NEUROBIOLOGY OF AGING (2012)

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Valosin-containing protein (VCP) mutations in sporadic amyotrophic lateral sclerosis

Yevgeniya Abramzon et al.

NEUROBIOLOGY OF AGING (2012)

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Mutational analysis of the VCP gene in Parkinson's disease

Elisa Majounie et al.

NEUROBIOLOGY OF AGING (2012)

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Mutation analysis of VCP in familial and sporadic amyotrophic lateral sclerosis

Kelly L. Williams et al.

NEUROBIOLOGY OF AGING (2012)

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VCP mutations in familial and sporadic amyotrophic lateral sclerosis

Max Koppers et al.

NEUROBIOLOGY OF AGING (2012)

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Novel mutation in VCP gene causes atypical amyotrophic lateral sclerosis

Paloma Gonzalez-Perez et al.

NEUROLOGY (2012)

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Inclusion Body Myopathy With Paget Disease of Bone and Frontotemporal Dementia Linked to VCP p.Arg155Cys in a Korean Family

Eun-Joo Kim et al.

ARCHIVES OF NEUROLOGY (2011)

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Decreases in valosin-containing protein result in increased levels of tau phosphorylated at Ser262/356

Philip J. Dolan et al.

FEBS LETTERS (2011)

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The Structural and Functional Basis of the p97/Valosin-containing Protein (VCP)-interacting Motif (VIM) MUTUALLY EXCLUSIVE BINDING OF COFACTORS TO THE N-TERMINAL DOMAIN OF p97

Petra Haenzelmann et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2011)

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Editorial Material Medicine, Research & Experimental

Another VCP interactor: NF is enough

Conrad C. Weihl

JOURNAL OF CLINICAL INVESTIGATION (2011)

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Molecular chaperones in protein folding and proteostasis

F. Ulrich Hartl et al.

NATURE (2011)

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Endolysosomal sorting of ubiquitylated caveolin-1 is regulated by VCP and UBXD1 and impaired by VCP disease mutations

Danilo Ritz et al.

NATURE CELL BIOLOGY (2011)

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The ubiquitin-selective segregase VCP/p97 orchestrates the response to DNA double-strand breaks

Mayura Meerang et al.

NATURE CELL BIOLOGY (2011)

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Amyloid-β and tau - a toxic pas de deux in Alzheimer's disease

Lars M. Ittner et al.

NATURE REVIEWS NEUROSCIENCE (2011)

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Derlin-1 is a rhomboid pseudoprotease required for the dislocation of mutant α-1 antitrypsin from the endoplasmic reticulum

Ethan J. Greenblatt et al.

NATURE STRUCTURAL & MOLECULAR BIOLOGY (2011)

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Editorial Material Clinical Neurology

NOVEL p.Ile151Val MUTATION IN VCP IN A PATIENT OF AFRICAN AMERICAN DESCENT WITH SPORADIC ALS

M. DeJesus-Hernandez et al.

NEUROLOGY (2011)

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Article Clinical Neurology

Distinct distal myopathy phenotype caused by VCP gene mutation in a Finnish family

Johanna Palmio et al.

NEUROMUSCULAR DISORDERS (2011)

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CHIP promotes the degradation of mutant SOD1 by reducing its interaction with VCP and S6/S6′ subunits of 26S proteasome

Jin-Sun Choi et al.

ANIMAL CELLS AND SYSTEMS (2010)

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Article Biochemistry & Molecular Biology

Imbalances in p97 co-factor interactions in human proteinopathy

Vanesa Fernandez-Saiz et al.

EMBO REPORTS (2010)

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Cdc48 and Ufd3, new partners of the ubiquitin protease Ubp3, are required for ribophagy

Batool Ossareh-Nazari et al.

EMBO REPORTS (2010)

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Inclusion body myopathy, Paget's disease of the bone and fronto-temporal dementia: a disorder of autophagy

Jeong-Sun Ju et al.

HUMAN MOLECULAR GENETICS (2010)

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Structure and Function of the PLAA/Ufd3-p97/Cdc48 Complex

Liyan Qiu et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2010)

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Valosin-containing Protein (VCP) in Novel Feedback Machinery between Abnormal Protein Accumulation and Transcriptional Suppression

Masaaki Koike et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2010)

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Editorial Material Biochemical Research Methods

Waltz, an exciting new move in amyloid prediction

Mikael Oliveberg

NATURE METHODS (2010)

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Exome Sequencing Reveals VCP Mutations as a Cause of Familial ALS

Jane O. Johnson et al.

NEURON (2010)

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The ERAD Inhibitor Eeyarestatin I Is a Bifunctional Compound with a Membrane-Binding Domain and a p97/VCP Inhibitory Group

Qiuyan Wang et al.

PLOS ONE (2010)

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Review Cell Biology

Polyglutamine Diseases: Where does Toxicity Come from? What is Toxicity? Where are We Going?

Toshiaki Takahashi et al.

JOURNAL OF MOLECULAR CELL BIOLOGY (2010)

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UBXD1 is a VCP-interacting protein that is involved in ER-associated degradation

Masami Nagahama et al.

BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS (2009)

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VCP Mutations Causing Frontotemporal Lobar Degeneration Disrupt Localization of TDP-43 and Induce Cell Death

Michael A. Gitcho et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2009)

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Article Cell Biology

Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP disease

Jeong-Sun Ju et al.

JOURNAL OF CELL BIOLOGY (2009)

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Article Geriatrics & Gerontology

Inclusion body myopathy and frontotemporal dementia caused by a novel VCP mutation

Anna Bersano et al.

NEUROBIOLOGY OF AGING (2009)

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Clinical outcome in 19 French and Spanish patients with valosin-containing protein myopathy associated with Paget's disease of bone and frontotemporal dementia

Tanya Stojkovic et al.

NEUROMUSCULAR DISORDERS (2009)

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Ubiquitin ligase Kf-1 is involved in the endoplasmic reticulum-associated degradation pathway

Yoshiaki Maruyama et al.

BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS (2008)

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Regulation of ER-associated degradation via p97/VCP-interacting motif

Petek Ballar et al.

BIOCHEMICAL SOCIETY TRANSACTIONS (2008)

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Autophagic-lysosomal perturbation enhances tau aggregation in transfectants with induced wild-type tau expression

Tadanori Hamano et al.

EUROPEAN JOURNAL OF NEUROSCIENCE (2008)

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Impaired ERAD and ER stress are early and specific events in polyglutamine toxicity

Martin L. Duennwald et al.

GENES & DEVELOPMENT (2008)

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An Italian family with inclusion-body myopathy and frontotemporal dementia due to mutation in the VCP gene

Teresa Gidaro et al.

MUSCLE & NERVE (2008)

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Cdc48/p97 promotes reformation of the nucleus by extracting the kinase Aurora B from chromatin

Kristijan Ramadan et al.

NATURE (2007)

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Identification of SVIP as an endogenous inhibitor of endoplasmic reticulum-associated degradation

Petek Ballar et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2007)

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Novel VCP mutations in inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia

G. D. J. Watts et al.

CLINICAL GENETICS (2007)

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Review Clinical Neurology

Valosin-containing protein and the pathogenesis of frontotemporal dementia associated with inclusion body myopathy

Jake B. Guinto et al.

ACTA NEUROPATHOLOGICA (2007)

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Studies on peptide:N-glycanase-p97 interaction suggest that p97 phosphorylation modulates endoplasmic reticulum-associated degradation

Gang Zhao et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2007)

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Article Cell Biology

The RBCC gene RFP2 (leu5) encodes a novel transmembrane E3 ubiquitin ligase involved in ERAD

Mikael Lerner et al.

MOLECULAR BIOLOGY OF THE CELL (2007)

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Pathological consequences of VCP mutations on human striated muscle

Christian U. Huebbers et al.

BRAIN (2007)

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APOE is a potential modifier gene in an autosomal dominant form of frontotemporal dementia (IBMPFD)

Sarju G. Mehta et al.

GENETICS IN MEDICINE (2007)

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ER E3 ubiquitin ligase HRD-1 and its specific partner chaperone BiP play important roles in ERAD and developmental growth in Caenorhabditis elegans

Yohei Sasagawa et al.

GENES TO CELLS (2007)

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P37 is a p97 adaptor required for Golgi and ER biogenesis in interphase and at the end of mitosis

Keiji Uchiyama et al.

DEVELOPMENTAL CELL (2006)

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Characterization of erasin (UBXD2): a new ER protein that promotes ER-associated protein degradation

Jing Liang et al.

JOURNAL OF CELL SCIENCE (2006)

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Ataxin-3 binds VCP/p97 and regulates retrotranslocation of ERAD substrates

Xiaoyan Zhong et al.

HUMAN MOLECULAR GENETICS (2006)

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Calcium-sensing receptor ubiquitination and degradation mediated by the E3 ubiquitin ligase dorfin

Y Huang et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2006)

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ATPase activity of p97/valosin-containing protein is regulated by oxidative modification of the evolutionally conserved cysteine 522 residue in walker a motif

M Noguchi et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2005)

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Article Clinical Neurology

Inclusion body myopathy and Paget disease is linked to a novel mutation in the VCP gene

D Haubenberger et al.

NEUROLOGY (2005)

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Ubx2 links the Cdc48 complex to ER-associated protein degradation

O Neuber et al.

NATURE CELL BIOLOGY (2005)

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Gp78, a membrane-anchored ubiquitin ligase, associates with Insig-1 and couples sterol-regulated ubiquitination to degradation of HMG CoA reductase

BL Song et al.

MOLECULAR CELL (2005)

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Article Biochemistry & Molecular Biology

Akt-mediated valosin-containing protein 97 phosphorylation regulates its association with ubiquitinated proteins

JB Klein et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2005)

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Control of α subunit of eukaryotic translation initiation factor 2 (eIF2α) phosphorylation by the human papillomavirus type 18 E6 oncoprotein:: Implications for eIF2α-dependent gene expression and cell death

S Kazemi et al.

MOLECULAR AND CELLULAR BIOLOGY (2004)

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Distinct roles for the AAA ATPases NSF and p97 in the secretory pathway

S Dalal et al.

MOLECULAR BIOLOGY OF THE CELL (2004)

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Article Biochemistry & Molecular Biology

Physical and functional interaction between Dorfin and Valosin-containing protein that are colocalized in ubiquitylated inclusions in neurodegenerative disorders

S Ishigaki et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2004)

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Article Biochemistry & Molecular Biology

AAA ATPase p97/valosin-containing protein interacts with gp78, a ubiquitin ligase for endoplasmic reticulum-associated degradation

XY Zhong et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2004)

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Article Biochemistry & Molecular Biology

Complete structure of p97/valosin-containing protein reveals communication between nucleotide domains

B DeLaBarre et al.

NATURE STRUCTURAL BIOLOGY (2003)

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D1 ring is stable and nucleotide-independent, whereas D2 ring undergoes major conformational changes during the ATPase cycle of p97-VCP

Q Wang et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2003)

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Vacuole-creating protein in neurodegenerative diseases in humans

Y Mizuno et al.

NEUROSCIENCE LETTERS (2003)

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ATPase activity of p97-valosin-containing protein (VCP) - D2 mediates the major enzyme activity, and D1 contributes to the heat-induced activity

CC Song et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2003)

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Article Biochemistry & Molecular Biology

AAA-ATPase p97/Cdc48p, a cytosolic chaperone required for endoplasmic reticulum-associated protein degradation

E Rabinovich et al.

MOLECULAR AND CELLULAR BIOLOGY (2002)

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Valosin-containing protein is a multiubiquitin chain targeting factor required in ubiquitin-proteasome degradation

RM Dai et al.

NATURE CELL BIOLOGY (2001)

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Article Biochemistry & Molecular Biology

Structure of the AAA ATPase p97

XD Zhang et al.

MOLECULAR CELL (2000)

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