Related references
Note: Only part of the references are listed.Transgenic mice expressing mutant forms VCP/p97 recapitulate the full spectrum of IBMPFD including degeneration in muscle, brain and bone
Sara K. Custer et al.
HUMAN MOLECULAR GENETICS (2010)
Cytoplasmic Mislocalization of TDP-43 Is Toxic to Neurons and Enhanced by a Mutation Associated with Familial Amyotrophic Lateral Sclerosis
Sami J. Barmada et al.
JOURNAL OF NEUROSCIENCE (2010)
Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS
Andrew C. Elden et al.
NATURE (2010)
TDP-43 transgenic mice develop spastic paralysis and neuronal inclusions characteristic of ALS and frontotemporal lobar degeneration
Hans Wils et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (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 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration
Iga Wegorzewska 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)
Mutations in the FUS/TLS Gene on Chromosome 16 Cause Familial Amyotrophic Lateral Sclerosis
T. J. Kwiatkowski et al.
SCIENCE (2009)
Prion protein aggresomes are poly(A)+ ribonucleoprotein complexes that induce a PKR-mediated deficient cell stress response
Kevin Goggin et al.
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH (2008)
Ribonucleoprotein complexes in neurologic diseases
Jernej Ule
CURRENT OPINION IN NEUROBIOLOGY (2008)
A90V TDP-43 variant results in the aberrant localization of TDP-43 in vitro
Matthew J. Winton et al.
FEBS LETTERS (2008)
Proteotoxic stress and inducible chaperone networks in neurodegenerative disease and aging
Richard I. Morimoto
GENES & DEVELOPMENT (2008)
Disturbance of nuclear and cytoplasmic TAR DNA-binding protein (TDP-43) induces disease-like redistribution, sequestration, and aggregate formation
Matthew J. Winton et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2008)
TDP-43, the signature protein of FTLD-U, is a neuronal activity-responsive factor
I. -Fan Wang et al.
JOURNAL OF NEUROCHEMISTRY (2008)
Oxysterol-binding protein-1 (OSBP1) modulates processing and trafficking of the amyloid precursor protein
Celina V. Zerbinatti et al.
MOLECULAR NEURODEGENERATION (2008)
Substrate-targeting γ-secretase modulators
Thomas L. Kukar et al.
NATURE (2008)
TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis
Jemeen Sreedharan et al.
SCIENCE (2008)
Stress granules: The Tao of RNA triage
Paul Anderson et al.
TRENDS IN BIOCHEMICAL SCIENCES (2008)
Severe subcortical TDP-43 pathology in sporadic frontotemporal lobar degeneration with motor neuron disease
Nicholas J. Brandmeir et al.
ACTA NEUROPATHOLOGICA (2008)
Progranulin mediates caspase-dependent cleavage of TAR DNA binding protein-43
Yong-Jie Zhang et al.
JOURNAL OF NEUROSCIENCE (2007)
TDP-43 in familial and sporadic frontotemporal lobar degeneration with ubiquitin inclusions
Nigel J. Cairns et al.
AMERICAN JOURNAL OF PATHOLOGY (2007)
Absence of heterogeneous nuclear ribonucleoproteins and survival motor neuron protein in TDP-43 positive inclusions in frontotemporal lobar degeneration
Manuela Neumann et al.
ACTA NEUROPATHOLOGICA (2007)
Ataxin-2 interacts with the DEAD/H-box RNA helicase DDX6 and interferes with P-bodies and stress granules
Ute Nonhoff et al.
MOLECULAR BIOLOGY OF THE CELL (2007)
Staufen- and FMRP-containing neuronal RNPs are structurally and functionally related to somatic P bodies
Scott A. Barbee et al.
NEURON (2006)
Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis
Manuela Neumann et al.
SCIENCE (2006)
TDP-43 binds heterogeneous nuclear ribonucleoprotein A/B through its C-terminal tail - An important region for the inhibition of cystic fibrosis transmembrane conductance regulator exon 9 splicing
E Buratti et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2005)
Depletion of TDP 43 overrides the need for exonic and intronic splicing enhancers in the human apoA-II gene
PA Mercado et al.
NUCLEIC ACIDS RESEARCH (2005)
An intronic polypyrimidine-rich element downstream of the donor site modulates cystic fibrosis transmembrane conductance regulator exon 9 alternative splicing
E Zuccato et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2004)
CHIP and Hsp70 regulate tau ubiquitination, degradation and aggregation
L Petrucelli et al.
HUMAN MOLECULAR GENETICS (2004)
CHIP-Hsc70 complex ubiquitinates phosphorylated tau and enhances cell survival
H Shimura et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2004)
Survival motor neuron protein facilitates assembly of stress granules
YM Hua et al.
FEBS LETTERS (2004)
Stress granule assembly is mediated by prion-like aggregation of TIA-1
N Gilks et al.
MOLECULAR BIOLOGY OF THE CELL (2004)
Nuclear factor TDP-43 binds to the polymorphic TG repeats in CFTR intron 8 and causes skipping of Exon 9: A functional link with disease penetrance
E Buratti et al.
AMERICAN JOURNAL OF HUMAN GENETICS (2004)
Stress granules: sites of mRNA triage that regulate mRNA stability and translatability
N Kedersha et al.
BIOCHEMICAL SOCIETY TRANSACTIONS (2002)
Inherent toxicity of aggregates implies a common mechanism for protein misfolding diseases
M Bucciantini et al.
NATURE (2002)
Stress proteins induced by arsenic
LM Del Razo et al.
TOXICOLOGY AND APPLIED PHARMACOLOGY (2001)
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)
Accumulation of mutant huntingtin fragments in aggresome-like inclusion bodies as a result of insufficient protein degradation
S Waelter et al.
MOLECULAR BIOLOGY OF THE CELL (2001)
Dynamic shuttling of TIA-1 accompanies the recruitment of mRNA to mammalian stress granules
N Kedersha et al.
JOURNAL OF CELL BIOLOGY (2000)