TDP-43 mutation in familial amyotrophic lateral sclerosis.
about
TARDBP mutation analysis in TDP-43 proteinopathies and deciphering the toxicity of mutant TDP-43Conjoint pathologic cascades mediated by ALS/FTLD-U linked RNA-binding proteins TDP-43 and FUSPhenotypic Heterogeneity of Monogenic Frontotemporal DementiaUltrastructural studies of ALS mitochondria connect altered function and permeability with defects of mitophagy and mitochondriogenesisMolecular motor proteins and amyotrophic lateral sclerosisRisk factors for amyotrophic lateral sclerosisHyperphosphorylation as a defense mechanism to reduce TDP-43 aggregationFUS transgenic rats develop the phenotypes of amyotrophic lateral sclerosis and frontotemporal lobar degenerationTDP-43 Proteinopathy and ALS: Insights into Disease Mechanisms and Therapeutic Targets.The complex molecular biology of amyotrophic lateral sclerosis (ALS)Identification of genetic modifiers of TDP-43 neurotoxicity in DrosophilaLoss of C9ORF72 impairs autophagy and synergizes with polyQ Ataxin-2 to induce motor neuron dysfunction and cell deathTDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration.Loss of murine TDP-43 disrupts motor function and plays an essential role in embryogenesis.Non-human primate model of amyotrophic lateral sclerosis with cytoplasmic mislocalization of TDP-43The dual functions of the extreme N-terminus of TDP-43 in regulating its biological activity and inclusion formation.Partial loss of TDP-43 function causes phenotypes of amyotrophic lateral sclerosis.Neuropathology of Amyotrophic Lateral Sclerosis and Its Variants.Pedigree with frontotemporal lobar degeneration--motor neuron disease and Tar DNA binding protein-43 positive neuropathology: genetic linkage to chromosome 9.TDP-43 expression in mouse models of amyotrophic lateral sclerosis and spinal muscular atrophy.Association between novel TARDBP mutations and Chinese patients with amyotrophic lateral sclerosisCytoplasmic mislocalization of TDP-43 is toxic to neurons and enhanced by a mutation associated with familial amyotrophic lateral sclerosis.Motor neuron expression of the voltage-gated calcium channel cacophony restores locomotion defects in a Drosophila, TDP-43 loss of function model of ALS.TDP-43 suppresses tau expression via promoting its mRNA instability.Identification of compounds protective against G93A-SOD1 toxicity for the treatment of amyotrophic lateral sclerosis.Multiplex SILAC analysis of a cellular TDP-43 proteinopathy model reveals protein inclusions associated with SUMOylation and diverse polyubiquitin chainsThe role of transactive response DNA-binding protein-43 in amyotrophic lateral sclerosis and frontotemporal dementia.Drosophila lines with mutant and wild type human TDP-43 replacing the endogenous gene reveals phosphorylation and ubiquitination in mutant lines in the absence of viability or lifespan defects.TDP-43 mediates degeneration in a novel Drosophila model of disease caused by mutations in VCP/p97.TAR DNA-binding protein 43 in neurodegenerative disease.Phosphorylated TDP-43 pathology and hippocampal sclerosis in progressive supranuclear palsyCytoplasmic accumulation and aggregation of TDP-43 upon proteasome inhibition in cultured neurons.The emerging use of in vivo optical imaging in the study of neurodegenerative diseases.Interaction with polyglutamine aggregates reveals a Q/N-rich domain in TDP-43.Frameshift and novel mutations in FUS in familial amyotrophic lateral sclerosis and ALS/dementiaAmyotrophic lateral sclerosis-associated proteins TDP-43 and FUS/TLS function in a common biochemical complex to co-regulate HDAC6 mRNA.TDP-43 proteinopathy in familial motor neurone disease with TARDBP A315T mutation: a case report.Knockdown of the Drosophila fused in sarcoma (FUS) homologue causes deficient locomotive behavior and shortening of motoneuron terminal branches.Alteration of POLDIP3 splicing associated with loss of function of TDP-43 in tissues affected with ALSGenetic rodent models of amyotrophic lateral sclerosis
P2860
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P2860
TDP-43 mutation in familial amyotrophic lateral sclerosis.
description
2008 nî lūn-bûn
@nan
2008年の論文
@ja
2008年学术文章
@wuu
2008年学术文章
@zh
2008年学术文章
@zh-cn
2008年学术文章
@zh-hans
2008年学术文章
@zh-my
2008年学术文章
@zh-sg
2008年學術文章
@yue
2008年學術文章
@zh-hant
name
TDP-43 mutation in familial amyotrophic lateral sclerosis.
@en
TDP-43 mutation in familial amyotrophic lateral sclerosis.
@nl
type
label
TDP-43 mutation in familial amyotrophic lateral sclerosis.
@en
TDP-43 mutation in familial amyotrophic lateral sclerosis.
@nl
prefLabel
TDP-43 mutation in familial amyotrophic lateral sclerosis.
@en
TDP-43 mutation in familial amyotrophic lateral sclerosis.
@nl
P2093
P356
P1433
P1476
TDP-43 mutation in familial amyotrophic lateral sclerosis.
@en
P2093
Akihide Koyama
Akio Yokoseki
Akira Tsujino
Akiyoshi Kakita
Asako Tagawa
Atsushi Shiga
Chun-Feng Tan
Hiroto Eguchi
Hiroyuki Kaneko
Hitoshi Takahashi
P304
P356
10.1002/ANA.21392
P577
2008-04-01T00:00:00Z