4.6 Review

The implications of physiological biomolecular condensates in amyotrophic lateral sclerosis

相关参考文献

注意:仅列出部分参考文献,下载原文获取全部文献信息。
Review Cell Biology

Regulation of ribosomal RNA gene copy number, transcription and nucleolus organization in eukaryotes

Yutaro Hori et al.

Summary: Ribosome, one of the earliest biological machineries, is crucial for protein synthesis and cellular function. Recent studies have revealed the mechanisms of maintaining the integrity and copy number of ribosomal DNA, the regulation of ribosomal RNA transcription, as well as the role of liquid-liquid phase separation in nucleolus function. The relationship between ribosomal RNA production, cell senescence, and disease is also discussed.

NATURE REVIEWS MOLECULAR CELL BIOLOGY (2023)

Article Multidisciplinary Sciences

FUS regulates a subset of snoRNA expression and modulates the level of rRNA modifications

Kishor Gawade et al.

Summary: FUS is a multifunctional protein involved in various aspects of RNA metabolism. Depletion of FUS leads to differential expression of snoRNAs, which guide the modification of rRNAs and snRNAs. Through our analysis, we observed hypermodification of rRNA species in cells with FUS knockout or FUS mutation related to ALS, suggesting a role of FUS in modulating rRNA modification and translation efficiency/fidelity. This study provides insights into a potential translation-related mechanism underlying ALS development and progression.

SCIENTIFIC REPORTS (2023)

Article Cell Biology

lncRNA NEAT1: Key player in neurodegenerative diseases

Kun Li et al.

Summary: Neurodegenerative diseases are a leading cause of disability worldwide, and the search for biomarkers and therapeutic targets is urgent. Long non-coding RNAs (lncRNAs), including NEAT1, have emerged as important regulators in these diseases. NEAT1 is upregulated in various neurodegenerative diseases, but its role in these diseases is still unclear. This review summarizes the current knowledge of NEAT1's association with misfolded protein aggregation in different neurodegenerative diseases and aims to inspire further research for more precise and effective treatments.

AGEING RESEARCH REVIEWS (2023)

Review Biochemistry & Molecular Biology

Liquid-Liquid Phase Separation of TDP-43 and FUS in Physiology and Pathology of Neurodegenerative Diseases

Jenny L. Carey et al.

Summary: Liquid-liquid phase separation of RNA-binding proteins plays a crucial role in cellular function, but aberrant phase transition can lead to protein aggregation and disease. This article reviews the factors that mediate and regulate phase separation of TDP-43 and FUS, and discusses the potential benefits and possible therapeutic strategies for restoring their functional phase in neuronal cells.

FRONTIERS IN MOLECULAR BIOSCIENCES (2022)

Article Biochemistry & Molecular Biology

Condensates induced by transcription inhibition localize active chromatin to nucleoli

Takaaki Yasuhara et al.

Summary: The proper function of the genome relies on the spatial organization of DNA, RNA, and proteins. Research shows that transcription inhibition leads to the formation of condensates (CITIs) that drastically alter the genome's spatial organization. Additionally, proper transcription and RNA processing prevent the generation of gene fusions caused by DNA double-strand breaks (DSBs).

MOLECULAR CELL (2022)

Review Neurosciences

Dysregulation of Translation in TDP-43 Proteinopathies: Deficits in the RNA Supply Chain and Local Protein Production

Reed T. Bjork et al.

Summary: Local control of gene expression is crucial for regulating development and function of the nervous system. TDP-43, an RNA binding protein, has been implicated in neurodegenerative disorders such as ALS and FTD. Dysregulation of cytoplasmic translation plays a significant role in the pathogenesis of these diseases. This review summarizes recent findings on the translational regulation of TDP-43 and its potential implications in various experimental models.

FRONTIERS IN NEUROSCIENCE (2022)

Article Cell Biology

Sequence Determinants of TDP-43 Ribonucleoprotein Condensate Formation and Axonal Transport in Neurons

Sonali S. Vishal et al.

Summary: This study identifies key structural elements, including the alpha-helical domain, phenylalanine residues within the LARKS, and the RGG motif, that are crucial for TDP-43 RNP transport and condensate formation in neurons. These findings suggest that these structural elements may mediate efficient recruitment of motors and adaptor proteins, and offer a possible mechanism underlying ALS-linked TDP-43 defects in axonal transport and homeostasis.

FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY (2022)

Review Biochemistry & Molecular Biology

Stress Granule Homeostasis, Aberrant Phase Transition, and Amyotrophic Lateral Sclerosis

Zhanxu Li et al.

Summary: This study summarizes the relationship between amyotrophic lateral sclerosis (ALS) and stress granules (SGs), and finds that ALS-related mutations may lead to the disease by disrupting SG homeostasis and affecting cytoplasmic phase equilibrium.

ACS CHEMICAL NEUROSCIENCE (2022)

Article Biology

Long non-coding RNA Neat1 and paraspeckle components are translational regulators in hypoxia

Anne-Claire Godet et al.

Summary: This study reveals the association between the paraspeckle, a stressed-induced nuclear body, and IRES-dependent translation. The long non-coding RNA Neat1 is identified as a key translational regulator, active on IRESs of (lymph)angiogenic and cardioprotective factor mRNAs. Paraspeckle proteins, including p54(nrb) and PSPC1, as well as nucleolin and RPS2, are found to be ITAFs for IRES subgroups. These findings highlight the importance of IRES-dependent translation during stress response.
Review Neurosciences

Stress granules in the spinal muscular atrophy and amyotrophic lateral sclerosis: The correlation and promising therapy

LiDan Hu et al.

Summary: This review provides an overview of the genetic and biochemical studies on stress granules in SMA and ALS, and discusses the mechanisms through which stress granules are involved in the pathogenesis of these diseases. It also summarizes the current therapies and proposes potential targets on stress granules for future interventions of SMA and ALS.

NEUROBIOLOGY OF DISEASE (2022)

Article Cell Biology

Nuclear RNA binding regulates TDP-43 nuclear localization and passive nuclear export

Lauren Duan et al.

Summary: The nuclear clearance of TDP-43 is a characteristic of neurodegenerative diseases. This study reveals that the nuclear export of TDP-43 is facilitated by the binding of nuclear RNAs, and the balance of transcription, pre-mRNA processing, and RNA export regulates the nuclear localization of TDP-43.

CELL REPORTS (2022)

Article Cell Biology

Hyperosmotic-stress-induced liquid-liquid phase separation of ALS-related proteins in the nucleus

Chao Gao et al.

Summary: This study investigates the effect of hyperosmotic stress on the liquid-liquid phase separation (LLPS) of amyotrophic lateral sclerosis (ALS)-related proteins. The results show that the dynamic and reversibility of FUS granules are sustained with increasing hypertonic stimulation time, while those of TDP-43 granules decrease significantly. FUS granules contain the essential chaperone Hsp40, capable of protecting amyloid proteins from aggregation. Additionally, FUS nuclear granules can co-localize with paraspeckles, but not with other nuclear bodies, while TDP-43 nuclear granules do not co-localize with any of the aforementioned nuclear bodies.

CELL REPORTS (2022)

Article Medicine, General & Internal

Trial of Antisense Oligonucleotide Tofersen for SOD1 ALS

Timothy M. Miller et al.

Summary: This study examined the use of tofersen in patients with SOD1 ALS and found that it can reduce protein concentrations in the cerebrospinal fluid and plasma, but does not improve clinical outcomes and is associated with adverse events. Further investigation is needed to evaluate the effects of early initiation of tofersen.

NEW ENGLAND JOURNAL OF MEDICINE (2022)

Article Biochemistry & Molecular Biology

Familial ALS-associated SFPQ variants promote the formation of SFPQ cytoplasmic aggregates in primary neurons

Jocelyn Widagdo et al.

Summary: SFPQ, a nuclear RNA-binding protein involved in physiological processes, is mislocalized and forms cytoplasmic aggregates in relation to ALS. Familial ALS-associated mutations in SFPQ enhance its binding to zinc and aggregation, leading to dysregulation of AMPA receptor subunit composition.

OPEN BIOLOGY (2022)

Review Pharmacology & Pharmacy

Opportunities for histone deacetylase inhibition in amyotrophic lateral sclerosis

Yvonne E. Klingl et al.

Summary: ALS is a devastating neurodegenerative disease with limited treatment options. Research suggests that interference with histone deacetylases may be helpful in treating ALS.

BRITISH JOURNAL OF PHARMACOLOGY (2021)

Review Cell Biology

The nucleolus as a multiphase liquid condensate

Denis L. J. Lafontaine et al.

Summary: The nucleolus is a membraneless organelle involved in ribonucleoprotein assembly, with recent studies suggesting that it forms as a biomolecular condensate via liquid-liquid phase separation (LLPS). Insights from LLPS studies are enhancing our understanding of the nucleolar structure-function relationship.

NATURE REVIEWS MOLECULAR CELL BIOLOGY (2021)

Review Biochemistry & Molecular Biology

Walking the line: mechanisms underlying directional mRNA transport and localisation in neurons and beyond

Reem Abouward et al.

Summary: Localisation of mRNA plays a crucial role in establishing and maintaining cellular polarity, synaptic plasticity, and other biological processes. Recent discoveries regarding RNA hitchhiking on cytoplasmic organelles have significant implications on our understanding of neuronal function in development, adulthood, and neurodegeneration. The mechanisms controlling mRNA localisation are largely conserved across evolution and have been extensively studied in various biological models.

CELLULAR AND MOLECULAR LIFE SCIENCES (2021)

Review Biochemistry & Molecular Biology

FUS and TDP-43 Phases in Health and Disease

Bede Portz et al.

Summary: This study discussed how the PrLDs of FUS and TDP-43 regulate phase transitions of condensates and their implications in both health and disease. It suggests that the emerging understanding of LLPS and aggregation of FUS and TDP-43 could be utilized to design new therapies for neurodegenerative diseases.

TRENDS IN BIOCHEMICAL SCIENCES (2021)

Article Multidisciplinary Sciences

Axonal TDP-43 condensates drive neuromuscular junction disruption through inhibition of local synthesis of nuclear encoded mitochondrial proteins

Topaz Altman et al.

Summary: Mislocalization of TDP-43 in axons of ALS patients leads to the assembly of G3BP1-positive RNP condensates, inhibiting local protein synthesis in distal axons and NMJs.

NATURE COMMUNICATIONS (2021)

Article Neurosciences

Nucleolar stress in C9orf72 and sporadic ALS spinal motor neurons precedes TDP-43 mislocalization

Olubankole Aladesuyi Arogundade et al.

Summary: Nucleolar stress, characterized by nucleolar shrinkage, occurs in C9-ALS spinal motor neurons before main pathological hallmarks appear. This phenomenon is also observed in SALS motor neurons, suggesting that nucleolar stress may be a significant and upstream issue in sporadic disease.

ACTA NEUROPATHOLOGICA COMMUNICATIONS (2021)

Article Neurosciences

Nucleolin Rescues TDP-43 Toxicity in Yeast and Human Cell Models

Caterina Peggion et al.

Summary: TDP-43 is a nuclear protein implicated in neurodegenerative disorders, with its misfolding and aggregation playing a key role in their etiopathology. Nucleolar protein nucleolin (NCL) has been identified as a potent suppressor of TDP-43 toxicity, alleviating its damage in both yeast and human cells. The findings suggest that NCL could promote TDP-43 nuclear retention, reducing the formation of toxic cytosolic TDP-43 inclusions.

FRONTIERS IN CELLULAR NEUROSCIENCE (2021)

Review Biochemistry & Molecular Biology

Connecting the dots: RNP granule network in health and disease

Haiyan An et al.

Summary: All cells contain membraneless RNP granules consisting of RNA and proteins, which play crucial roles in organizing cellular processes. The dynamic nature of RNP granules allows cells to adapt to different functional states and environments. Disruption of the RNP granule network may contribute to diseases such as ALS.

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH (2021)

Review Biochemistry & Molecular Biology

It's not just a phase: function and characteristics of RNA-binding proteins in phase separation

Hannah J. Wiedner et al.

Summary: The organization of RNA in phase-separated condensates provides insights into how sequestration of RNA-binding proteins and RNAs modulates gene expression. This understanding has implications for basic cell biology, the pathogenesis of human diseases, and the development of novel therapies. Biomolecular condensates formed through phase separation play a crucial role in coordinating cellular reactions and regulating various biological phenomena.

NATURE STRUCTURAL & MOLECULAR BIOLOGY (2021)

Article Cell Biology

TOR signaling regulates liquid phase separation of the SMN complex governing snRNP biogenesis

Maximilian Schilling et al.

Summary: The study found that mTOR and ribosomal protein S6 kinase beta-1 are key regulators of the SMN complex's ability to condense in Cajal bodies, and phosphorylation is an important regulatory mechanism for the complex's activity.

CELL REPORTS (2021)

Article Multidisciplinary Sciences

FUS-ALS mutants alter FMRP phase separation equilibrium and impair protein translation

Nicol Birsa et al.

Summary: Mutations in FUS lead to its mislocalization in the cytoplasm, affecting the sequestration and repression of translation of another RBP, FMRP, in motor neurons associated with ALS.

SCIENCE ADVANCES (2021)

Review Cell Biology

Aberrant Stress Granule Dynamics and Aggrephagy in ALS Pathogenesis

Yi Zhang et al.

Summary: Cells regulate the relationship between stress granules and protein aggregates through the autophagy pathway to maintain cellular homeostasis. Defective aggrephagy is associated with various neurodegenerative diseases, including ALS.
Article Multidisciplinary Sciences

HSP70 chaperones RNA-free TDP-43 into anisotropic intranuclear liquid spherical shells

Haiyang Yu et al.

Summary: This study found that the RNA binding protein TDP-43 forms intranuclear or cytoplasmic aggregates in age-related neurodegenerative diseases. Through mathematical modeling, the primary components of TDP-43 liquid core were identified to be HSP70 family chaperones, and these structures converted to aggregates when ATP levels were reduced.

SCIENCE (2021)

Review Clinical Neurology

TDP-43 proteinopathies: a new wave of neurodegenerative diseases

Eva Maria Johanna de Boer et al.

Summary: TDP-43 is a crucial protein involved in the pathogenesis of various neurodegenerative diseases, including ALS and FTLD. Understanding the molecular pathways through which TDP-43 mediates neurodegeneration is essential for the development of effective therapies.

JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY (2021)

Article Clinical Neurology

Characteristic Features of FUS Inclusions in Spinal Motor Neurons of Sporadic Amyotrophic Lateral Sclerosis

Kensuke Ikenaka et al.

JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY (2020)

Review Neurosciences

The Overlapping Genetics of Amyotrophic Lateral Sclerosis and Frontotemporal Dementia

Yevgeniya A. Abramzon et al.

FRONTIERS IN NEUROSCIENCE (2020)

Review Neurosciences

Dysregulation of RNA-Binding Proteins in Amyotrophic Lateral Sclerosis

Yuan Chao Xue et al.

FRONTIERS IN MOLECULAR NEUROSCIENCE (2020)

Editorial Material Multidisciplinary Sciences

Formation of liquid-like cellular organelles depends on their composition

Chiu Fan Lee

NATURE (2020)

Article Multidisciplinary Sciences

Composition-dependent thermodynamics of intracellular phase separation

Joshua A. Riback et al.

NATURE (2020)

Article Neurosciences

UsnRNP trafficking is regulated by stress granules and compromised by mutant ALS proteins

Simona Rossi et al.

NEUROBIOLOGY OF DISEASE (2020)

Article Clinical Neurology

TDP-43 transports ribosomal protein mRNA to regulate axonal local translation in neuronal axons

Seiichi Nagano et al.

ACTA NEUROPATHOLOGICA (2020)

Review Neurosciences

The role of TDP-43 mislocalization in amyotrophic lateral sclerosis

Terry R. Suk et al.

MOLECULAR NEURODEGENERATION (2020)

Review Neurosciences

The Neuromuscular Junction in Health and Disease: Molecular Mechanisms Governing Synaptic Formation and Homeostasis

Pedro M. Rodriguez Cruz et al.

FRONTIERS IN MOLECULAR NEUROSCIENCE (2020)

Article Biochemistry & Molecular Biology

Cross-Regulation between TDP-43 and Paraspeckles Promotes Pluripotency-Differentiation Transition

Miha Modic et al.

MOLECULAR CELL (2019)

Review Biochemistry & Molecular Biology

Local translation in neurons: visualization and function

Christine E. Holt et al.

NATURE STRUCTURAL & MOLECULAR BIOLOGY (2019)

Article Multidisciplinary Sciences

The nucleolus functions as a phase-separated protein quality control compartment

F. Frottin et al.

SCIENCE (2019)

Review Neurosciences

Stress granules and neurodegeneration

Benjamin Wolozin et al.

NATURE REVIEWS NEUROSCIENCE (2019)

Review Neurosciences

ALS Genetics, Mechanisms, and Therapeutics: Where Are We Now?

Rita Mejzini et al.

FRONTIERS IN NEUROSCIENCE (2019)

Review Biochemistry & Molecular Biology

RGG/RG Motif Regions in RNA Binding and Phase Separation

P. Andrew Chong et al.

JOURNAL OF MOLECULAR BIOLOGY (2018)

Article Biochemistry & Molecular Biology

Functional Domains of NEAT1 Architectural lncRNA Induce Paraspeckle Assembly through Phase Separation

Tomohiro Yamazaki et al.

MOLECULAR CELL (2018)

Article Neurosciences

Protective paraspeckle hyper-assembly downstream of TDP-43 loss of function in amyotrophic lateral sclerosis

Tatyana A. Shelkovnikova et al.

MOLECULAR NEURODEGENERATION (2018)

Article Multidisciplinary Sciences

Self-interaction of NPM1 modulates multiple mechanisms of liquid-liquid phase separation

Diana M. Mitrea et al.

NATURE COMMUNICATIONS (2018)

Article Multidisciplinary Sciences

A complex of C9ORF72 and p62 uses arginine methylation to eliminate stress granules by autophagy

Maneka Chitiprolu et al.

NATURE COMMUNICATIONS (2018)

Article Multidisciplinary Sciences

Active nuclear import and passive nuclear export are the primary determinants of TDP-43 localization

Emile S. Pinarbasi et al.

SCIENTIFIC REPORTS (2018)

Article Multidisciplinary Sciences

Nuclear egress of TDP-43 and FUS occurs independently of Exportin-1/CRM1

Helena Ederle et al.

SCIENTIFIC REPORTS (2018)

Article Multidisciplinary Sciences

Axonal G3BP1 stress granule protein limits axonal mRNA translation and nerve regeneration

Pabitra K. Sahoo et al.

NATURE COMMUNICATIONS (2018)

Review Biochemistry & Molecular Biology

TDP-43 and FUS en route from the nucleus to the cytoplasm

Helena Ederle et al.

FEBS LETTERS (2017)

Article Biochemistry & Molecular Biology

Heat Shock-induced Phosphorylation of TAR DNA-binding Protein 43 (TDP-43) by MAPK/ERK Kinase Regulates TDP-43 Function

Wen Li et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2017)

Review Medicine, Research & Experimental

New perspectives of physiological and pathological functions of nucleolin (NCL)

Wenyu Jia et al.

LIFE SCIENCES (2017)

Article Multidisciplinary Sciences

Therapeutic reduction of ataxin-2 extends lifespan and reduces pathology in TDP-43 mice

Lindsay A. Becker et al.

NATURE (2017)

Article Biochemistry & Molecular Biology

NEAT1 scaffolds RNA-binding proteins and the Microprocessor to globally enhance pri-miRNA processing

Li Jiang et al.

NATURE STRUCTURAL & MOLECULAR BIOLOGY (2017)

Article Multidisciplinary Sciences

Amyotrophic lateral sclerosis-linked mutations increase the viscosity of liquid-like TDP-43 RNP granules in neurons

Pallavi P. Gopal et al.

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

Editorial Material Biochemistry & Molecular Biology

Special focus on the Cajal Body

Karla M. Neugebauer

RNA BIOLOGY (2017)

Article Multidisciplinary Sciences

HDAC6 inhibition reverses axonal transport defects in motor neurons derived from FUS-ALS patients

Wenting Guo et al.

NATURE COMMUNICATIONS (2017)

Article Biochemistry & Molecular Biology

NEAT1 scaffolds RNA-binding proteins and the Microprocessor to globally enhance pri-miRNA processing

Li Jiang et al.

NATURE STRUCTURAL & MOLECULAR BIOLOGY (2017)

Article Biochemistry & Molecular Biology

Minor intron splicing is regulated by FUS and affected by ALS-associated FUS mutants

Stefan Reber et al.

EMBO JOURNAL (2016)

Article Cell Biology

Structural, super-resolution microscopy analysis of paraspeckle nuclear body organization

Jason A. West et al.

JOURNAL OF CELL BIOLOGY (2016)

Review Biochemistry & Molecular Biology

Physiological functions and pathobiology of TDP-43 and FUS/TLS proteins

Antonia Ratti et al.

JOURNAL OF NEUROCHEMISTRY (2016)

Article Biology

Distinct stages in stress granule assembly and disassembly

Joshua R. Wheeler et al.

Article Biochemistry & Molecular Biology

A Liquid-to-Solid Phase Transition of the ALS Protein FUS Accelerated by Disease Mutation

Avinash Patel et al.

Article Neurosciences

Alterations in stress granule dynamics driven by TDP-43 and FUS: a link to pathological inclusions in ALS?

Anaïs Aulas et al.

Frontiers in Cellular Neuroscience (2015)

Article Multidisciplinary Sciences

An acetylation switch controls TDP-43 function and aggregation propensity

Todd J. Cohen et al.

NATURE COMMUNICATIONS (2015)

Article Neurosciences

FUS/TLS deficiency causes behavioral and pathological abnormalities distinct from amyotrophic lateral sclerosis

Yoshihiro Kino et al.

ACTA NEUROPATHOLOGICA COMMUNICATIONS (2015)

Review Cell Biology

Liquid-Liquid Phase Separation in Biology

Anthony A. Hyman et al.

ANNUAL REVIEW OF CELL AND DEVELOPMENTAL BIOLOGY, VOL 30 (2014)

Article Biochemistry & Molecular Biology

Reactive nucleolar and Cajal body responses to proteasome inhibition in sensory ganglion neurons

Ana Palanca et al.

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE (2014)

Review Neurosciences

Gem Depletion: Amyotrophic Lateral Sclerosis and Spinal Muscular Atrophy Crossover

Ruben J. Cauchi

CNS NEUROSCIENCE & THERAPEUTICS (2014)

Article Biochemistry & Molecular Biology

The Mechanistic Links Between Proteasome Activity, Aging and Agerelated Diseases

Isabel Saez et al.

CURRENT GENOMICS (2014)

Article Biochemistry & Molecular Biology

Compromised paraspeckle formation as a pathogenic factor in FUSopathies

Tatyana A. Shelkovnikova et al.

HUMAN MOLECULAR GENETICS (2014)

Article Neurosciences

ALS-Linked Mutations Enlarge TDP-43-Enriched Neuronal RNA Granules in the Dendritic Arbor

LiQun Liu-Yesucevitz et al.

JOURNAL OF NEUROSCIENCE (2014)

Article Clinical Neurology

Nuclear localization of SMN and FUS is not altered in fibroblasts from patients with sporadic ALS

Shingo Kariya et al.

AMYOTROPHIC LATERAL SCLEROSIS AND FRONTOTEMPORAL DEGENERATION (2014)

Review Clinical Neurology

Protein aggregation in amyotrophic lateral sclerosis

Anna M. Blokhuis et al.

ACTA NEUROPATHOLOGICA (2013)

Article Biochemistry & Molecular Biology

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

J. Ross Buchan et al.

Article Medicine, Research & Experimental

Spliceosome integrity is defective in the motor neuron diseases ALS and SMA

Hitomi Tsuiji et al.

EMBO MOLECULAR MEDICINE (2013)

Review Biochemistry & Molecular Biology

Defining the RGG/RG Motif

Palaniraja Thandapani et al.

MOLECULAR CELL (2013)

Article Biochemistry & Molecular Biology

Alternative 3′-end processing of long noncoding RNA initiates construction of nuclear paraspeckles

Takao Naganuma et al.

EMBO JOURNAL (2012)

Article Multidisciplinary Sciences

TDP-43 promotes microRNA biogenesis as a component of the Drosha and Dicer complexes

Yukio Kawahara et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2012)

Article Cell Biology

FUS-SMN Protein Interactions Link the Motor Neuron Diseases ALS and SMA

Tomohiro Yamazaki et al.

CELL REPORTS (2012)

Article Biochemistry & Molecular Biology

Identification of Neuronal RNA Targets of TDP-43-containing Ribonucleoprotein Complexes

Chantelle F. Sephton et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2011)

Article Neurosciences

Characterizing the RNA targets and position-dependent splicing regulation by TDP-43

James R. Tollervey et al.

NATURE NEUROSCIENCE (2011)

Article Biochemistry & Molecular Biology

RNA targets of wild-type and mutant FET family proteins

Jessica I. Hoell et al.

NATURE STRUCTURAL & MOLECULAR BIOLOGY (2011)

Article Biochemistry & Molecular Biology

Mutant FUS proteins that cause amyotrophic lateral sclerosis incorporate into stress granules

Daryl A. Bosco et al.

HUMAN MOLECULAR GENETICS (2010)

Article Multidisciplinary Sciences

Altered distributions of Gemini of coiled bodies and mitochondria in motor neurons of TDP-43 transgenic mice

Xiu Shan et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2010)

Article Biochemistry & Molecular Biology

TDP-43 is recruited to stress granules in conditions of oxidative insult

Claudia Colombrita et al.

JOURNAL OF NEUROCHEMISTRY (2009)

Article Biochemistry & Molecular Biology

TDP-43 localizes in mRNA transcription and processing sites in mammalian neurons

Inigo Casafont et al.

JOURNAL OF STRUCTURAL BIOLOGY (2009)

Article Biochemistry & Molecular Biology

An Architectural Role for a Nuclear Noncoding RNA: NEAT1 RNA Is Essential for the Structure of Paraspeckles

Christine M. Clemson et al.

MOLECULAR CELL (2009)

Article Multidisciplinary Sciences

Mutations in the FUS/TLS Gene on Chromosome 16 Cause Familial Amyotrophic Lateral Sclerosis

T. J. Kwiatkowski et al.

SCIENCE (2009)

Article Cell Biology

Structural determinants of the cellular localization and shuttling of TDP-43

Youhna M. Ayala et al.

JOURNAL OF CELL SCIENCE (2008)

Article Biochemistry & Molecular Biology

Microtubule acetylation promotes kinesin-1 binding and transport

Nathan A. Reed et al.

CURRENT BIOLOGY (2006)

Article Biochemistry & Molecular Biology

Egulating gene expression through RNA nuclear retention

KV Prasanth et al.

Article Cell Biology

Cajal body proteins SMN and coilin show differential dynamic behaviour in vivo

JE Sleeman et al.

JOURNAL OF CELL SCIENCE (2003)

Review Cell Biology

From fixed to FRAP: measuring protein mobility and activity in living cells

EAJ Reits et al.

NATURE CELL BIOLOGY (2001)

Review Medicine, General & Internal

Medical progress: Amyotrophic lateral sclerosis.

LP Rowland et al.

NEW ENGLAND JOURNAL OF MEDICINE (2001)