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Amanda Atanasio et al.
SCIENTIFIC REPORTS (2016)
Therapeutic effect of berberine on TDP-43-related pathogenesis in FTLD and ALS
Cheng-Fu Chang et al.
JOURNAL OF BIOMEDICAL SCIENCE (2016)
Distribution of dipeptide repeat proteins in cellular models and C9orf72 mutation cases suggests link to transcriptional silencing
Martin H. Schludi et al.
ACTA NEUROPATHOLOGICA (2015)
A Liquid-to-Solid Phase Transition of the ALS Protein FUS Accelerated by Disease Mutation
Avinash Patel et al.
CELL (2015)
Liquids, Fibers, and Gels: The Many Phases of Neurodegeneration
Shana Elbaum-Garfinkle et al.
DEVELOPMENTAL CELL (2015)
ALS mutant FUS proteins are recruited into stress granules in induced pluripotent stem cell-derived motoneurons
J. Lenzi et al.
Disease Models & Mechanisms (2015)
Unsaturated fatty acid-induced non-canonical autophagy: unusual? or unappreciated?
Vytas A. Bankaitis
EMBO JOURNAL (2015)
Nucleolar stress and impaired stress granule formation contribute to C9orf72 RAN translation-induced cytotoxicity
Zhouteng Tao et al.
HUMAN MOLECULAR GENETICS (2015)
Long Term Aggresome Accumulation Leads to DNA Damage, p53-dependent Cell Cycle Arrest, and Steric Interference in Mitosis
Meng Lu et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2015)
TDP-43 is intercellularly transmitted across axon terminals
Marisa S. Feiler et al.
JOURNAL OF CELL BIOLOGY (2015)
SOD1 protein aggregates stimulate macropinocytosis in neurons to facilitate their propagation
Rafaa Zeineddine et al.
MOLECULAR NEURODEGENERATION (2015)
NEURODEGENERATION Problems at the nuclear pore
Bennett W. Fox et al.
NATURE (2015)
The C9orf72 repeat expansion disrupts nucleocytoplasmic transport
Ke Zhang et al.
NATURE (2015)
GGGGCC repeat expansion in C9orf72 compromises nucleocytoplasmic transport
Brian D. Freibaum et al.
NATURE (2015)
Modifiers of C9orf72 dipeptide repeat toxicity connect nucleocytoplasmic transport defects to FTD/ALS
Ana Jovicic et al.
NATURE NEUROSCIENCE (2015)
Spreading of pathology in neurodegenerative diseases: a focus on human studies
Johannes Brettschneider et al.
NATURE REVIEWS NEUROSCIENCE (2015)
ALS/FTD Mutation-Induced Phase Transition of FUS Liquid Droplets and Reversible Hydrogels into Irreversible Hydrogels Impairs RNP Granule Function
Tetsuro Murakami et al.
NEURON (2015)
Structural and kinetic analysis of protein-aggregate strains in vivo using binary epitope mapping
Johan Bergh et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2015)
Supersaturation is a major driving force for protein aggregation in neurodegenerative diseases
Prajwal Ciryam et al.
TRENDS IN PHARMACOLOGICAL SCIENCES (2015)
From nucleation to widespread propagation: A prion-like concept for ALS
Zuzanna Maniecka et al.
VIRUS RESEARCH (2015)
Exposure to ALS-FTD-CSF generates TDP-43 aggregates in glioblastoma cells through exosomes and TNTs-like structure
Xuebing Ding et al.
ONCOTARGET (2015)
GGGGCC microsatellite RNA is neuritically localized, induces branching defects, and perturbs transport granule function
Alondra Schweizer Burguete et al.
eLife (2015)
A fly model for the CCUG-repeat expansion of myotonic dystrophy type 2 reveals a novel interaction with MBNL1
Zhenming Yu et al.
HUMAN MOLECULAR GENETICS (2015)
Development and evaluation of a clinical staging system for amyotrophic lateral sclerosis
Adriano Chio et al.
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY (2015)
Rapamycin and Chloroquine: The In Vitro and In Vivo Effects of Autophagy-Modifying Drugs Show Promising Results in Valosin Containing Protein Multisystem Proteinopathy
Angele Nalbandian et al.
PLOS ONE (2015)
Lysosomal Storage Diseases-Regulating Neurodegeneration
Rob U. Onyenwoke et al.
JOURNAL OF EXPERIMENTAL NEUROSCIENCE (2015)
Biological properties of extracellular vesicles and their physiological functions
Maria Yanez-Mo et al.
JOURNAL OF EXTRACELLULAR VESICLES (2015)
SOD1 silencing in motoneurons or glia rescues neuromuscular function in ALS mice
Elisabeth Dirren et al.
ANNALS OF CLINICAL AND TRANSLATIONAL NEUROLOGY (2015)
Experimental transmissibility of mutant SOD1 motor neuron disease
Jacob I. Ayers et al.
ACTA NEUROPATHOLOGICA (2014)
Liquid-Liquid Phase Separation in Biology
Anthony A. Hyman et al.
ANNUAL REVIEW OF CELL AND DEVELOPMENTAL BIOLOGY, VOL 30 (2014)
Structural characterization of the minimal segment of TDP-43 competent for aggregation
Miguel Mompean et al.
ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS (2014)
Autophagy modulates SNCA/α-synuclein release, thereby generating a hostile microenvironment
Anne-Maria Poehler et al.
AUTOPHAGY (2014)
The Mechanistic Links Between Proteasome Activity, Aging and Agerelated Diseases
Isabel Saez et al.
CURRENT GENOMICS (2014)
Motoneuron firing in amyotrophic lateral sclerosis (ALS)
Mamede de Carvalho et al.
FRONTIERS IN HUMAN NEUROSCIENCE (2014)
Widespread spinal cord transduction by intrathecal injection of rAAV delivers efficacious RNAi therapy for amyotrophic lateral sclerosis
Hongyan Wang et al.
HUMAN MOLECULAR GENETICS (2014)
C9ORF72, implicated in amytrophic lateral sclerosis and frontotemporal dementia, regulates endosomal trafficking
Manal A. Farg et al.
HUMAN MOLECULAR GENETICS (2014)
Intranuclear Aggregation of Mutant FUS/TLS as a Molecular Pathomechanism of Amyotrophic Lateral Sclerosis
Takao Nomura et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2014)
Differential roles of the ubiquitin proteasome system and autophagy in the clearance of soluble and aggregated TDP-43 species
Emma L. Scotter et al.
JOURNAL OF CELL SCIENCE (2014)
Proteomic analysis of cerebrospinal fluid extracellular vesicles: A comprehensive dataset
Davide Chiasserini et al.
JOURNAL OF PROTEOMICS (2014)
Intra-axonal protein aggregation in the peripheral nervous system
Anne Vital et al.
JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM (2014)
C9orf72 nucleotide repeat structures initiate molecular cascades of disease
Aaron R. Haeusler et al.
NATURE (2014)
Autophagy induction enhances TDP43 turnover and survival in neuronal ALS models
Sami J. Barmada et al.
NATURE CHEMICAL BIOLOGY (2014)
ALS-dying forward, backward or outward?
Mark R. Baker
NATURE REVIEWS NEUROLOGY (2014)
The phenotypic variability of amyotrophic lateral sclerosis
Bart Swinnen et al.
NATURE REVIEWS NEUROLOGY (2014)
The role of FUS gene variants in neurodegenerative diseases
Hao Deng et al.
NATURE REVIEWS NEUROLOGY (2014)
MicroRNA-206: A Potential Circulating Biomarker Candidate for Amyotrophic Lateral Sclerosis
Janne M. Toivonen et al.
PLOS ONE (2014)
Extracellular-vesicle type of volume transmission and tunnelling-nanotube type of wiring transmission add a new dimension to brain neuro-glial networks
Luigi F. Agnati et al.
PHILOSOPHICAL TRANSACTIONS OF THE ROYAL SOCIETY B-BIOLOGICAL SCIENCES (2014)
Intercellular propagated misfolding of wild-type Cu/Zn superoxide dismutase occurs via exosome-dependent and -independent mechanisms
Leslie I. Grad et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2014)
C9orf72 repeat expansions cause neurodegeneration in Drosophila through arginine-rich proteins
Sarah Mizielinska et al.
SCIENCE (2014)
A seeded propagation of Cu, Zn-superoxide dismutase aggregates in amyotrophic lateral sclerosis
Mariko Ogawa et al.
FRONTIERS IN CELLULAR NEUROSCIENCE (2014)
Motor neurons and the generation of spinal motor neuron diversity
Nicolas Stifani
FRONTIERS IN CELLULAR NEUROSCIENCE (2014)
Limited role of free TDP-43 as a diagnostic tool in neurodegenerative diseases
Emily Feneberg et al.
AMYOTROPHIC LATERAL SCLEROSIS AND FRONTOTEMPORAL DEGENERATION (2014)
Protein aggregation in amyotrophic lateral sclerosis
Anna M. Blokhuis et al.
ACTA NEUROPATHOLOGICA (2013)
C9orf72 frontotemporal lobar degeneration is characterised by frequent neuronal sense and antisense RNA foci
Sarah Mizielinska et al.
ACTA NEUROPATHOLOGICA (2013)
Antisense transcripts of the expanded C9ORF72 hexanucleotide repeat form nuclear RNA foci and undergo repeat-associated non-ATG translation in c9FTD/ALS
Tania F. Gendron et al.
ACTA NEUROPATHOLOGICA (2013)
Loss of Function of C9orf72 Causes Motor Deficits in a Zebrafish Model of Amyotrophic Lateral Sclerosis
Sorana Ciura et al.
ANNALS OF NEUROLOGY (2013)
Trehalose delays the progression of amyotrophic lateral sclerosis by enhancing autophagy in motoneurons
Karen Castillo et al.
AUTOPHAGY (2013)
Co-induction of the heat shock response ameliorates disease progression in a mouse model of human spinal and bulbar muscular atrophy: implications for therapy
Bilal Malik et al.
BRAIN (2013)
Loss of TDP-43 causes age-dependent progressive motor neuron degeneration
Yohei Iguchi et al.
BRAIN (2013)
Intracellular seeded aggregation of mutant Cu,Zn-superoxide dismutase associated with amyotrophic lateral sclerosis
Yoshiaki Furukawa et al.
FEBS LETTERS (2013)
Expression of wild-type human superoxide dismutase-1 in mice causes amyotrophic lateral sclerosis
Karin S. Graffmo et al.
HUMAN MOLECULAR GENETICS (2013)
ALS mutant FUS disrupts nuclear localization and sequesters wild-type FUS within cytoplasmic stress granules
Caroline Vance et al.
HUMAN MOLECULAR GENETICS (2013)
Mutant Copper-Zinc Superoxide Dismutase (SOD1) Induces Protein Secretion Pathway Alterations and Exosome Release in Astrocytes IMPLICATIONS FOR DISEASE SPREADING AND MOTOR NEURON PATHOLOGY IN AMYOTROPHIC LATERAL SCLEROSIS
Manuela Basso et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2013)
The Disease-associated r(GGGGCC)n Repeat from the C9orf72 Gene Forms Tract Length-dependent Uni- and Multimolecular RNA G-quadruplex Structures
Kaalak Reddy et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2013)
Stress granules as crucibles of ALS pathogenesis
Yun R. Li et al.
JOURNAL OF CELL BIOLOGY (2013)
Extracellular vesicles: Exosomes, microvesicles, and friends
Graca Raposo et al.
JOURNAL OF CELL BIOLOGY (2013)
The LIR motif - crucial for selective autophagy
Asa Birna Birgisdottir et al.
JOURNAL OF CELL SCIENCE (2013)
An antisense oligonucleotide against SOD1 delivered intrathecally for patients with SOD1 familial amyotrophic lateral sclerosis: a phase 1, randomised, first-in-man study (vol 12, pg 435, 2013)
T. M. Miller et al.
LANCET NEUROLOGY (2013)
Genetics of amyotrophic lateral sclerosis: an update
Sheng Chen et al.
MOLECULAR NEURODEGENERATION (2013)
Therapeutic AAV9-mediated Suppression of Mutant SOD1 Slows Disease Progression and Extends Survival in Models of Inherited ALS
Kevin D. Foust et al.
MOLECULAR THERAPY (2013)
Autophagosomes form at ER-mitochondria contact sites
Maho Hamasaki et al.
NATURE (2013)
RNA Toxicity from the ALS/FTD C9ORF72 Expansion Is Mitigated by Antisense Intervention
Christopher J. Donnelly et al.
NEURON (2013)
Characterizing TDP-43 interaction with its RNA targets
Amit Bhardwaj et al.
NUCLEIC ACIDS RESEARCH (2013)
Deletion of C9ORF72 Results in Motor Neuron Degeneration and Stress Sensitivity in C-elegans
Martine Therrien et al.
PLOS ONE (2013)
Loss of ALS-associated TDP-43 in zebrafish causes muscle degeneration, vascular dysfunction, and reduced motor neuron axon outgrowth
Bettina Schmid et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2013)
Targeted degradation of sense and antisense C9orf72 RNA foci as therapy for ALS and frontotemporal degeneration
Clotilde Lagier-Tourenne et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2013)
Extracellular microvesicles from astrocytes contain functional glutamate transporters: regulation by protein kinase C and cell activation
Romain-Daniel Gosselin et al.
FRONTIERS IN CELLULAR NEUROSCIENCE (2013)
C9orf72 mutation is rare in Alzheimer's disease, Parkinson's disease, and essential tremor in China
Bin Jiao et al.
FRONTIERS IN CELLULAR NEUROSCIENCE (2013)
Prion-like Properties of Pathological TDP-43 Aggregates from Diseased Brains
Takashi Nonaka et al.
CELL REPORTS (2013)
Hexanucleotide Repeats in ALS/FTD Form Length-Dependent RNA Foci, Sequester RNA Binding Proteins, and Are Neurotoxic
Youn-Bok Lee et al.
CELL REPORTS (2013)
Neurotransmitter-Triggered Transfer of Exosomes Mediates Oligodendrocyte-Neuron Communication
Carsten Fruehbeis et al.
PLOS BIOLOGY (2013)
Eye tracking communication devices in amyotrophic lateral sclerosis: Impact on disability and quality of life
Marco Caligari et al.
AMYOTROPHIC LATERAL SCLEROSIS AND FRONTOTEMPORAL DEGENERATION (2013)
Yeast genetic screen reveals novel therapeutic strategy for ALS
Matthew D. Figley et al.
RARE DISEASES (2013)
An antisense oligonucleotide against SOD1 delivered intrathecally for patients with SOD1 familial amyotrophic lateral sclerosis: a phase 1, randomised, first-in-man study
Timothy M. Miller et al.
LANCET NEUROLOGY (2013)
Multivesicular Body Morphogenesis
Phyllis I. Hanson et al.
ANNUAL REVIEW OF CELL AND DEVELOPMENTAL BIOLOGY, VOL 28 (2012)
Young-onset amyotrophic lateral sclerosis: historical and other observations
Martin R. Turner et al.
BRAIN (2012)
Arginine methylation next to the PY-NLS modulates Transportin binding and nuclear import of FUS
Dorothee Dormann et al.
EMBO JOURNAL (2012)
Cellular Model of TAR DNA-binding Protein 43 (TDP-43) Aggregation Based on Its C-terminal Gln/Asn-rich Region
Mauricio Budini et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2012)
Can regional spreading of amyotrophic lateral sclerosis motor symptoms be explained by prion-like propagation?
Tadashi Kanouchi et al.
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY (2012)
Contrasting Pathology of the Stress Granule Proteins TIA-1 and G3BP in Tauopathies
Tara Vanderweyde et al.
JOURNAL OF NEUROSCIENCE (2012)
A C9orf72 promoter repeat expansion in a Flanders-Belgian cohort with disorders of the frontotemporal lobar degeneration-amyotrophic lateral sclerosis spectrum: a gene identification study
Ilse Gijselinck et al.
LANCET NEUROLOGY (2012)
Gains or losses: molecular mechanisms of TDP43-mediated neurodegeneration
Edward B. Lee et al.
NATURE REVIEWS NEUROSCIENCE (2012)
FUS-NLS/Transportin 1 Complex Structure Provides Insights into the Nuclear Targeting Mechanism of FUS and the Implications in ALS
Chunyan Niu et al.
PLOS ONE (2012)
Autophagy activators rescue and alleviate pathogenesis of a mouse model with proteinopathies of the TAR DNA-binding protein 43
I-Fang Wang et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2012)
Dysfunction of constitutive and inducible ubiquitin-proteasome system in amyotrophic lateral sclerosis: Implication for protein aggregation and immune response
Caterina Bendotti et al.
PROGRESS IN NEUROBIOLOGY (2012)
Pathological α-Synuclein Transmission Initiates Parkinson-like Neurodegeneration in Nontransgenic Mice
Kelvin C. Luk et al.
SCIENCE (2012)
Tunneling-nanotube direction determination in neurons and astrocytes
X. Sun et al.
CELL DEATH & DISEASE (2012)
C9orf72 hexanucleotide repeat associated with amyotrophic lateral sclerosis and frontotemporal dementia forms RNA G-quadruplexes
Pietro Fratta et al.
SCIENTIFIC REPORTS (2012)
Glial nuclear aggregates of superoxide dismutase-1 are regularly present in patients with amyotrophic lateral sclerosis
Karin Forsberg et al.
ACTA NEUROPATHOLOGICA (2011)
Elevated CSF TDP-43 levels in amyotrophic lateral sclerosis: Specificity, sensitivity, and a possible prognostic value
Yu-Ichi Noto et al.
AMYOTROPHIC LATERAL SCLEROSIS (2011)
Identification of compounds protective against G93A-SOD1 toxicity for the treatment of amyotrophic lateral sclerosis
Radhia Benmohamed et al.
AMYOTROPHIC LATERAL SCLEROSIS (2011)
Protein Folding and Modification in the Mammalian Endoplasmic Reticulum
Ineke Braakman et al.
ANNUAL REVIEW OF BIOCHEMISTRY, VOL 80 (2011)
The Seeds of Neurodegeneration: Prion-like Spreading in ALS
Magdalini Polymenidou et al.
CELL (2011)
TDP-43 regulates its mRNA levels through a negative feedback loop
Youhna M. Ayala et al.
EMBO JOURNAL (2011)
miRNAs as serum biomarkers for Duchenne muscular dystrophy
Davide Cacchiarelli et al.
EMBO MOLECULAR MEDICINE (2011)
A Seeding Reaction Recapitulates Intracellular Formation of Sarkosyl-insoluble Transactivation Response Element (TAR) DNA-binding Protein-43 Inclusions
Yoshiaki Furukawa et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2011)
The Dying-Back Phenomenon of Motor Neurons in ALS
Michal Dadon-Nachum et al.
JOURNAL OF MOLECULAR NEUROSCIENCE (2011)
Misfolded superoxide dismutase-1 in CSF from amyotrophic lateral sclerosis patients
Per Zetterstrom et al.
JOURNAL OF NEUROCHEMISTRY (2011)
Spinal Inhibitory Interneuron Pathology Follows Motor Neuron Degeneration Independent of Glial Mutant Superoxide Dismutase 1 Expression in SOD1-ALS Mice
Mehdi Hossaini et al.
JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY (2011)
Proteomics of human cerebrospinal fluid: Discovery and verification of biomarker candidates in neurodegenerative diseases using quantitative proteomics
A. C. Kroksveen et al.
JOURNAL OF PROTEOMICS (2011)
Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43
Magdalini Polymenidou et al.
NATURE NEUROSCIENCE (2011)
Mechanisms and Function of Dendritic Exocytosis
Matthew J. Kennedy et al.
NEURON (2011)
Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS
Mariely DeJesus-Hernandez et al.
NEURON (2011)
A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTD
Alan E. Renton et al.
NEURON (2011)
Cytoplasmic Accumulation and Aggregation of TDP-43 upon Proteasome Inhibition in Cultured Neurons
Janet van Eersel et al.
PLOS ONE (2011)
RNA-binding proteins with prion-like domains in ALS and FTLD-U
Aaron D. Gitler et al.
PRION (2011)
Non-ATG-initiated translation directed by microsatellite expansions
Tao Zu et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2011)
Astrocytes carrying the superoxide dismutase 1 (SOD1G93A) mutation induce wild-type motor neuron degeneration in vivo
Sophia T. Papadeas et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2011)
A yeast functional screen predicts new candidate ALS disease genes
Julien Couthouis et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2011)
Intermolecular transmission of superoxide dismutase 1 misfolding in living cells
Leslie I. Grad et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2011)
Prion-like propagation of mutant superoxide dismutase-1 misfolding in neuronal cells
Christian Muench et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2011)
Staging Alzheimer's disease progression with multimodality neuroimaging
Michael Ewers et al.
PROGRESS IN NEUROBIOLOGY (2011)
SOD1 Transcriptional and Posttranscriptional Regulation and Its Potential Implications in ALS
Pamela Milani et al.
NEUROLOGY RESEARCH INTERNATIONAL (2011)
Pattern of spread and prognosis in lower limb-onset ALS
Martin R. Turner et al.
AMYOTROPHIC LATERAL SCLEROSIS (2010)
TDP-43 in skeletal muscle of patients affected with amyotrophic lateral sclerosis
Gianni Soraru et al.
AMYOTROPHIC LATERAL SCLEROSIS (2010)
Ubiquitin/proteasome pathway impairment in neurodegeneration: therapeutic implications
Qian Huang et al.
APOPTOSIS (2010)
Microtubules govern stress granule mobility and dynamics
Elena S. Nadezhdina et al.
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH (2010)
Nuclear factor TDP-43 can affect selected microRNA levels
Emanuele Buratti et al.
FEBS JOURNAL (2010)
Effects of Gender in Amyotrophic Lateral Sclerosis
Pamela A. McCombe et al.
GENDER MEDICINE (2010)
Clinical progression of moderate-to-severe Alzheimer's disease and caregiver burden: a 12-month multicenter prospective observational study
Luis Agueera-Ortiz et al.
INTERNATIONAL PSYCHOGERIATRICS (2010)
Intracerebroventricular infusion of monoclonal antibody or its derived Fab fragment against misfolded forms of SOD1 mutant delays mortality in a mouse model of ALS
Francois Gros-Louis et al.
JOURNAL OF NEUROCHEMISTRY (2010)
Membrane budding and scission by the ESCRT machinery: it's all in the neck
James H. Hurley et al.
NATURE REVIEWS MOLECULAR CELL BIOLOGY (2010)
Regulation of Mammalian Autophagy in Physiology and Pathophysiology
Brinda Ravikumar et al.
PHYSIOLOGICAL REVIEWS (2010)
Superoxide Dismutase 1 and tgSOD1G93A Mouse Spinal Cord Seed Fibrils, Suggesting a Propagative Cell Death Mechanism in Amyotrophic Lateral Sclerosis
Ruth Chia et al.
PLOS ONE (2010)
Tar DNA Binding Protein-43 (TDP-43) Associates with Stress Granules: Analysis of Cultured Cells and Pathological Brain Tissue
Liqun Liu-Yesucevitz et al.
PLOS ONE (2010)
The multiple roles of TDP-43 in pre-mRNA processing and gene expression regulation
Emanuele Buratti et al.
RNA BIOLOGY (2010)
Increased TDP-43 protein in cerebrospinal fluid of patients with amyotrophic lateral sclerosis
Takashi Kasai et al.
ACTA NEUROPATHOLOGICA (2009)
Nerve Injection of Viral Vectors Efficiently Transfers Transgenes into Motor Neurons and Delivers RNAi Therapy Against ALS
Rui Wu et al.
ANTIOXIDANTS & REDOX SIGNALING (2009)
A Systematic Survey Identifies Prions and Illuminates Sequence Features of Prionogenic Proteins
Simon Alberti et al.
CELL (2009)
Prion protein misfolding and disease
Roger A. Moore et al.
CURRENT OPINION IN STRUCTURAL BIOLOGY (2009)
Phosphorylated and ubiquitinated TDP-43 pathological inclusions in ALS and FTLD-U are recapitulated in SH-SY5Y cells
Takashi Nonaka et al.
FEBS LETTERS (2009)
Mutations in TDP-43 link glycine-rich domain functions to amyotrophic lateral sclerosis
G. Scott Pesiridis et al.
HUMAN MOLECULAR GENETICS (2009)
Microenvironmental pH Is a Key Factor for Exosome Traffic in Tumor Cells
Isabella Parolini et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2009)
TDP-43 Is Intrinsically Aggregation-prone, and Amyotrophic Lateral Sclerosis-linked Mutations Accelerate Aggregation and Increase Toxicity
Brian S. Johnson et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2009)
Non-cell autonomous toxicity in neurodegenerative disorders: ALS and beyond
Hristelina Ilieva et al.
JOURNAL OF CELL BIOLOGY (2009)
TDP-43 is recruited to stress granules in conditions of oxidative insult
Claudia Colombrita et al.
JOURNAL OF NEUROCHEMISTRY (2009)
Beclin 1 Gene Transfer Activates Autophagy and Ameliorates the Neurodegenerative Pathology in α-Synuclein Models of Parkinson's and Lewy Body Diseases
Brian Spencer et al.
JOURNAL OF NEUROSCIENCE (2009)
Membrane scission by the ESCRT-III complex
Thomas Wollert et al.
NATURE (2009)
Prions hijack tunnelling nanotubes for intercellular spread
Karine Gousset et al.
NATURE CELL BIOLOGY (2009)
A role for motoneuron subtype-selective ER stress in disease manifestations of FALS mice
Smita Saxena et al.
NATURE NEUROSCIENCE (2009)
Natural history and clinical features of the flail arm and flail leg ALS variants
L. C. Wijesekera et al.
NEUROLOGY (2009)
Neurodegenerative Diseases Target Large-Scale Human Brain Networks
William W. Seeley et al.
NEURON (2009)
Structural insights into TDP-43 in nucleic-acid binding and domain interactions
Pan-Hsien Kuo et al.
NUCLEIC ACIDS RESEARCH (2009)
Amyotrophic lateral sclerosis
Lokesh C. Wijesekera et al.
ORPHANET JOURNAL OF RARE DISEASES (2009)
Design and construction of diverse mammalian prion strains
David W. Colby et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2009)
Aberrant cleavage of TDP-43 enhances aggregation and cellular toxicity
Yong-Jie Zhang et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2009)
Mechanisms of Prion Protein Aggregation
Sarah N. Fontaine et al.
PROTEIN AND PEPTIDE LETTERS (2009)
MicroRNA-206 Delays ALS Progression and Promotes Regeneration of Neuromuscular Synapses in Mice
Andrew H. Williams et al.
SCIENCE (2009)
Mutations in the FUS/TLS Gene on Chromosome 16 Cause Familial Amyotrophic Lateral Sclerosis
T. J. Kwiatkowski et al.
SCIENCE (2009)
Multivesicular Endosome Biogenesis in the Absence of ESCRTs
Susanne Stuffers et al.
TRAFFIC (2009)
Sporadic amyotrophic lateral sclerosis: two pathological patterns shown by analysis of distribution of TDP-43-immunoreactive neuronal and glial cytoplasmic inclusions
Yasushi Nishihira et al.
ACTA NEUROPATHOLOGICA (2008)
Phosphorylated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis
Masato Hasegawa et al.
ANNALS OF NEUROLOGY (2008)
TDP-43 in Cerebrospinal Fluid of Patients With Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis
Petra Steinacker et al.
ARCHIVES OF NEUROLOGY (2008)
Kinetic and thermodynamic stability of bacterial intracellular aggregates
Alba Espargaro et al.
FEBS LETTERS (2008)
ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1
Hideki Nishitoh et al.
GENES & DEVELOPMENT (2008)
The art of cellular communication: tunneling nanotubes bridge the divide
Steffen Gurke et al.
HISTOCHEMISTRY AND CELL BIOLOGY (2008)
Diagnosis and management of Parkinson's disease dementia
W. Poewe et al.
INTERNATIONAL JOURNAL OF CLINICAL PRACTICE (2008)
Therapeutic gene silencing delivered by a chemically modified small interfering RNA against mutant SOD1 slows amyotrophic lateral sclerosis progression
Hongyan Wang et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2008)
Late stage treatment with arimoclomol delays disease progression and prevents protein aggregation in the SOD1G93A mouse model of ALS
Bernadett Kalmar et al.
JOURNAL OF NEUROCHEMISTRY (2008)
TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis
Edor Kabashi et al.
NATURE GENETICS (2008)
Membrane nanotubes: dynamic long-distance connections between animal cells
Daniel M. Davis et al.
NATURE REVIEWS MOLECULAR CELL BIOLOGY (2008)
The dorsal motor nucleus of the vagus is not an obligatory trigger site of Parkinson's disease:: a critical analysis of α-synuclein staging
M. E. Kalaitzakis et al.
NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY (2008)
A yeast TDP-43 proteinopathy model: Exploring the molecular determinants of TDR-43 aggregation and cellular toxicity
Brian S. Johnson et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2008)
Regulation of heat shock protein 70 release in astrocytes: Role of signaling kinases
Anna R. Taylor et al.
DEVELOPMENTAL NEUROBIOLOGY (2007)
Oligodendrocytes secrete exosomes containing major myelin and stress-protective proteins:: Trophic support for axons?
Eva-Maria Kraemer-Albers et al.
PROTEOMICS CLINICAL APPLICATIONS (2007)
The prion strain phenomenon: Molecular basis and unprecedented features
Rodrigo Morales et al.
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE (2007)
Tryptophan 32 potentiates aggregation and cytotoxicity of a copper/zinc superoxide dismutase mutant associated with familial amyotrophic lateral sclerosis
David M. Taylor et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2007)
Focality of upper and lower motor neuron degeneration at the clinical onset of ALS
John Ravits et al.
NEUROLOGY (2007)
Life on the edge: a link between gene expression levels and aggregation rates of human proteins
Gian Gaetano Tartaglia et al.
TRENDS IN BIOCHEMICAL SCIENCES (2007)
Exosome-mediated transfer of mRNAs and microRNAs is a novel mechanism of genetic exchange between cells
Hadi Valadi et al.
NATURE CELL BIOLOGY (2007)
TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis
Tetsuaki Arai et al.
BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS (2006)
Structure, folding, and misfolding of Cu,Zn superoxide dismutase in amyotrophic lateral sclerosis
Rishi Rakhit et al.
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE (2006)
Immunoreactivities of p62, an ubiqutin-binding protein, in the spinal anterior horn cells of patients with amyotrophic lateral sclerosis
Yuji Mizuno et al.
JOURNAL OF THE NEUROLOGICAL SCIENCES (2006)
Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis
Manuela Neumann et al.
SCIENCE (2006)
Antisense oligonucleotide therapy for neurodegenerative disease
Richard A. Smith et al.
JOURNAL OF CLINICAL INVESTIGATION (2006)
hnRNP A1 relocalization to the stress granules reflects a role in the stress response
Sonia Guil et al.
MOLECULAR AND CELLULAR BIOLOGY (2006)
Spinal cord endoplasmic reticulum stress associated with a microsomal accumulation of mutant superoxide dismutase-1 in an ALS model
H Kikuchi et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2006)
Chromogranin-mediated secretion of mutant superoxide dismutase proteins linked to amyotrophic lateral sclerosis
M Urushitani et al.
NATURE NEUROSCIENCE (2006)
Extracellular heat shock protein 70: A critical component for motoneuron survival
MB Robinson et al.
JOURNAL OF NEUROSCIENCE (2005)
The pathobiology of amyotrophic lateral sclerosis: A proteinopathy?
MJ Strong et al.
JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY (2005)
Molecular and cellular pathways of neurodegeneration in motor neurone disease
PJ Shaw
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY (2005)
Stress granules and processing bodies are dynamically linked sites of mRNP remodeling
N Kedersha et al.
JOURNAL OF CELL BIOLOGY (2005)
Impairment of starvation-induced and constitutive autophagy in Atg7-deficient mice
M Komatsu et al.
JOURNAL OF CELL BIOLOGY (2005)
Impaired extracellular secretion of mutant superoxide dismutase 1 associates with neurotoxicity in familial amyotrophic lateral sclerosis
BJ Turner et al.
JOURNAL OF NEUROSCIENCE (2005)
Curcumin has potent anti-amyloidogenic effects for Alzheimer's β-amyloid fibrils in vitro
K Ono et al.
JOURNAL OF NEUROSCIENCE RESEARCH (2004)
Roles of molecular chaperones in protein misfolding diseases
JM Barral et al.
SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY (2004)
Amyotrophic lateral sclerosis is a distal axonopathy: evidence in mice and man
LR Fischer et al.
EXPERIMENTAL NEUROLOGY (2004)
Survival motor neuron protein facilitates assembly of stress granules
YM Hua et al.
FEBS LETTERS (2004)
Protein aggregation and neurodegenerative disease
CA Ross et al.
NATURE MEDICINE (2004)
Familial amyotrophic lateral sclerosis mutants of copper/zinc superoxide dismutase are susceptible to disulfide reduction
A Tiwari et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2003)
Characterization and functional implications of the RNA binding properties of nuclear factor TDP-43, a novel splicing regulator of CFTR exon 9
E Buratti et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2001)
Protacs: Chimeric molecules that target proteins to the Skp1-Cullin-F box complex for ubiquitination and degradation
KM Sakamoto et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2001)
Medical progress: Amyotrophic lateral sclerosis.
LP Rowland et al.
NEW ENGLAND JOURNAL OF MEDICINE (2001)
Macromolecular crowding: an important but neglected aspect of the intracellular environment
RJ Ellis
CURRENT OPINION IN STRUCTURAL BIOLOGY (2001)
Dynamic shuttling of TIA-1 accompanies the recruitment of mRNA to mammalian stress granules
N Kedersha et al.
JOURNAL OF CELL BIOLOGY (2000)
Adaptation and selection of prion protein strain conformations following interspecies transmission of transmissible mink encephalopathy
JC Bartz et al.
JOURNAL OF VIROLOGY (2000)