Related references
Note: Only part of the references are listed.TDP-43 pathology and neuronal loss in amyotrophic lateral sclerosis spinal cord
Johannes Brettschneider et al.
ACTA NEUROPATHOLOGICA (2014)
SQSTM1 mutations - Bridging Paget disease of bone and ALS/FTLD
Sarah L. Rea et al.
EXPERIMENTAL CELL RESEARCH (2014)
Misfolded Polyglutamine, Polyalanine, and Superoxide Dismutase 1 Aggregate via Distinct Pathways in the Cell
Saskia Polling 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)
FUS is sequestered in nuclear aggregates in ALS patient fibroblasts
Jacob C. Schwartz et al.
MOLECULAR BIOLOGY OF THE CELL (2014)
Mutations in the Matrin 3 gene cause familial amyotrophic lateral sclerosis
Janel O. Johnson et al.
NATURE NEUROSCIENCE (2014)
The amyloid state and its association with protein misfolding diseases
Tuomas P. J. Knowles et al.
NATURE REVIEWS MOLECULAR CELL BIOLOGY (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)
Eukaryotic Stress Granules Are Cleared by Autophagy and Cdc48/VCP Function
J. Ross Buchan et al.
CELL (2013)
Recruitment into stress granules prevents irreversible aggregation of FUS protein mislocalized to the cytoplasm
Tatyana A. Shelkovnikova et al.
CELL CYCLE (2013)
The small heat shock proteins αB-crystallin and Hsp27 suppress SOD1 aggregation in vitro
Justin J. Yerbury et al.
CELL STRESS & CHAPERONES (2013)
Spliceosome integrity is defective in the motor neuron diseases ALS and SMA
Hitomi Tsuiji et al.
EMBO MOLECULAR MEDICINE (2013)
Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS
Hong Joo Kim et al.
NATURE (2013)
Coexistence of Huntington's disease and amyotrophic lateral sclerosis: a clinicopathologic study
Mari Tada et al.
ACTA NEUROPATHOLOGICA (2012)
Mechanisms of disease in frontotemporal lobar degeneration: gain of function versus loss of function effects
Glenda Halliday et al.
ACTA NEUROPATHOLOGICA (2012)
Cell-free Formation of RNA Granules: Low Complexity Sequence Domains Form Dynamic Fibers within Hydrogels
Masato Kato et al.
CELL (2012)
Indirect inhibition of 26S proteasome activity in a cellular model of Huntington's disease
Mark S. Hipp et al.
JOURNAL OF CELL BIOLOGY (2012)
Mutations in the profilin 1 gene cause familial amyotrophic lateral sclerosis
Chi-Hong Wu et al.
NATURE (2012)
Compartmentalization of superoxide dismutase 1 (SOD1G93A) aggregates determines their toxicity
Sarah J. Weisberg et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2012)
Aggregation of the 35-kDa fragment of TDP-43 causes formation of cytoplasmic inclusions and alteration of RNA processing
Mei-Xia Che et al.
FASEB JOURNAL (2011)
A Two-hit Hypothesis for Inclusion Formation by Carboxyl-terminal Fragments of TDP-43 Protein Linked to RNA Depletion and Impaired Microtubule-dependent Transport
G. Scott Pesiridis et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2011)
Cyclosporin-A-induced prion protein aggresomes are dynamic quality-control cellular compartments
Tziona Ben-Gedalya et al.
JOURNAL OF CELL SCIENCE (2011)
Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia
Han-Xiang Deng et al.
NATURE (2011)
Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS
Mariely DeJesus-Hernandez et al.
NEURON (2011)
Molecular Determinants and Genetic Modifiers of Aggregation and Toxicity for the ALS Disease Protein FUS/TLS
Zhihui Sun et al.
PLOS BIOLOGY (2011)
FUS and TARDBP but Not SOD1 Interact in Genetic Models of Amyotrophic Lateral Sclerosis
Edor Kabashi et al.
PLOS GENETICS (2011)
ANS Binding Reveals Common Features of Cytotoxic Amyloid Species
Benedetta Bolognesi et al.
ACS CHEMICAL BIOLOGY (2010)
Ubiquitin/proteasome pathway impairment in neurodegeneration: therapeutic implications
Qian Huang et al.
APOPTOSIS (2010)
TDP-43 Redistribution is an Early Event in Sporadic Amyotrophic Lateral Sclerosis
Maria Teresa Giordana et al.
BRAIN PATHOLOGY (2010)
Conformation Sensors that Distinguish Monomeric Proteins from Oligomers in Live Cells
Yasmin M. Ramdzan et al.
CHEMISTRY & BIOLOGY (2010)
Protein Aggregation and Defective RNA Metabolism as Mechanisms for Motor Neuron Damage
N. Ticozzi et al.
CNS & NEUROLOGICAL DISORDERS-DRUG TARGETS (2010)
FUS mutations in amyotrophic lateral sclerosis: clinical, pathological, neurophysiological and genetic analysis
Ian P. Blair et al.
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY (2010)
Mutations of optineurin in amyotrophic lateral sclerosis
Hirofumi Maruyama et al.
NATURE (2010)
Exome Sequencing Reveals VCP Mutations as a Cause of Familial ALS
Jane O. Johnson et al.
NEURON (2010)
The RNA-binding protein FUS/TLS is a common aggregate-interacting protein in polyglutamine diseases
Hiroshi Doi et al.
NEUROSCIENCE RESEARCH (2010)
The C-Terminal TDP-43 Fragments Have a High Aggregation Propensity and Harm Neurons by a Dominant-Negative Mechanism
Chunxing Yang et al.
PLOS ONE (2010)
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)
TDP-43 is consistently co-localized with ubiquitinated inclusions in sporadic and Guam amyotrophic lateral sclerosis but not in familial amyotrophic lateral sclerosis with and without SOD1 mutations
Satomi Maekawa et al.
NEUROPATHOLOGY (2009)
Mutations in FUS, an RNA Processing Protein, Cause Familial Amyotrophic Lateral Sclerosis Type 6
Caroline Vance 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)
ALS and FTLD: two faces of TDP-43 proteinopathy
R. M. Liscic et al.
EUROPEAN JOURNAL OF NEUROLOGY (2008)
Misfolded proteins partition between two distinct quality control compartments
Daniel Kaganovich et al.
NATURE (2008)
TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis
Edor Kabashi et al.
NATURE GENETICS (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)
TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis
Jemeen Sreedharan et al.
SCIENCE (2008)
Dissection of the autophagosome maturation process by a novel reporter protein, tandem fluorescent-tagged LC3
Shunsuke Kimura et al.
AUTOPHAGY (2007)
TDP-43 proteinopathy: the neuropathology underlying major forms of sporadic and familial frontotemporal lobar degeneration and motor neuron disease
Linda K. Kwong et al.
ACTA NEUROPATHOLOGICA (2007)
Pathological TDP-43 distinguishes sporadic amyotrophic lateral sclerosis from amyotrophic lateral sclerosis with SOD1 mutations
Ian R. A. Mackenzie et al.
ANNALS OF NEUROLOGY (2007)
ALIS are stress-induced protein storage compartments for substrates of the proteasome and autophagy
Jason Szeto et al.
AUTOPHAGY (2006)
DNA damage triggers nucleotide excision repair-dependent monoubiquitylation of histone H2A
Steven Bergink et al.
GENES & DEVELOPMENT (2006)
The pathobiology of amyotrophic lateral sclerosis: A proteinopathy?
MJ Strong et al.
JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY (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)
A mutation in the vesicle-trafficking protein VAPB causes late-onset spinal muscular atrophy and amyotrophic lateral sclerosis
AL Nishimura et al.
AMERICAN JOURNAL OF HUMAN GENETICS (2004)
Dorfin ubiquitylates mutant SOD1 and prevents mutant SOD1-mediated neurotoxicity
J Niwa et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2002)
Requirement of an intact microtubule cytoskeleton for aggregation and inclusion body formation by a mutant huntingtin fragment
PJ Muchowski et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2002)
A novel quality control compartment derived from the endoplasmic reticulum
S Kamhi-Nesher et al.
MOLECULAR BIOLOGY OF THE CELL (2001)
Intracellular aggregation of polypeptides with expanded polyglutamine domain is stimulated by stress-activated kinase MEKK1
AB Meriin et al.
JOURNAL OF CELL BIOLOGY (2001)
Cellular defenses against unfolded proteins: A cell biologist thinks about neurodegenerative diseases
MY Sherman et al.
NEURON (2001)
Aggresomes, inclusion bodies and protein aggregation
RR Kopito
TRENDS IN CELL BIOLOGY (2000)
Formation of high molecular weight complexes of mutant Cu,Zn-superoxide dismutase in a mouse model for familial amyotrophic lateral sclerosis
JA Johnston et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2000)
Share Paper
