Dysregulation of the ALS-associated gene TDP-43 leads to neuronal death and degeneration in mice.
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The emerging science of BMAA: do cyanobacteria contribute to neurodegenerative disease?Redox signalling directly regulates TDP-43 via cysteine oxidation and disulphide cross-linkingAltered ribostasis: RNA-protein granules in degenerative disordersA seeding reaction recapitulates intracellular formation of Sarkosyl-insoluble transactivation response element (TAR) DNA-binding protein-43 inclusionsConjoint pathologic cascades mediated by ALS/FTLD-U linked RNA-binding proteins TDP-43 and FUSFrom animal models to human disease: a genetic approach for personalized medicine in ALSThe extreme N-terminus of TDP-43 mediates the cytoplasmic aggregation of TDP-43 and associated toxicity in vivoTDP-43: a new player on the AD field?Pathological mechanisms underlying TDP-43 driven neurodegeneration in FTLD-ALS spectrum disordersMouse models of frontotemporal dementiaConverging mechanisms in ALS and FTD: disrupted RNA and protein homeostasisGenetic dissection of a cell-autonomous neurodegenerative disorder: lessons learned from mouse models of Niemann-Pick disease type CDoes a loss of TDP-43 function cause neurodegeneration?Ataxin-2 regulates RGS8 translation in a new BAC-SCA2 transgenic mouse modelTDP-43 Proteinopathy and ALS: Insights into Disease Mechanisms and Therapeutic Targets.Systems biology of neurodegenerative diseasesExendin-4 ameliorates motor neuron degeneration in cellular and animal models of amyotrophic lateral sclerosisInactivation of CDK/pRb pathway normalizes survival pattern of lymphoblasts expressing the FTLD-progranulin mutation c.709-1G>AIdentification of genetic modifiers of TDP-43 neurotoxicity in DrosophilaNon-human primate model of amyotrophic lateral sclerosis with cytoplasmic mislocalization of TDP-43ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43.Astrocyte pathology and the absence of non-cell autonomy in an induced pluripotent stem cell model of TDP-43 proteinopathy.Selective forelimb impairment in rats expressing a pathological TDP-43 25 kDa C-terminal fragment to mimic amyotrophic lateral sclerosis.Overexpression of ALS-associated p.M337V human TDP-43 in mice worsens disease features compared to wild-type human TDP-43 mice.Drosophila TDP-43 dysfunction in glia and muscle cells cause cytological and behavioural phenotypes that characterize ALS and FTLDExpression of ALS-linked TDP-43 mutant in astrocytes causes non-cell-autonomous motor neuron death in ratsPrevention of intestinal obstruction reveals progressive neurodegeneration in mutant TDP-43 (A315T) mice.Wild type human TDP-43 potentiates ALS-linked mutant TDP-43 driven progressive motor and cortical neuron degeneration with pathological features of ALS.Inhibition of TDP-43 accumulation by bis(thiosemicarbazonato)-copper complexes.Astrocytic TDP-43 pathology in Alexander diseaseMitochondrial dysfunction and decrease in body weight of a transgenic knock-in mouse model for TDP-43.RNA metabolism in neurodegenerative disease.Profiling the genes affected by pathogenic TDP-43 in astrocytesAbnormalities of Mitochondrial Dynamics in Neurodegenerative Diseases.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.Nuclear TAR DNA-binding protein 43: A new target for amyotrophic lateral sclerosis treatmentDual vulnerability of TDP-43 to calpain and caspase-3 proteolysis after neurotoxic conditions and traumatic brain injuryThe aggregation and neurotoxicity of TDP-43 and its ALS-associated 25 kDa fragment are differentially affected by molecular chaperones in Drosophila.Early retinal neurodegeneration and impaired Ran-mediated nuclear import of TDP-43 in progranulin-deficient FTLDRoles of ataxin-2 in pathological cascades mediated by TAR DNA-binding protein 43 (TDP-43) and Fused in Sarcoma (FUS)
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P2860
Dysregulation of the ALS-associated gene TDP-43 leads to neuronal death and degeneration in mice.
description
2011 nî lūn-bûn
@nan
2011 թուականի Յունուարին հրատարակուած գիտական յօդուած
@hyw
2011 թվականի հունվարին հրատարակված գիտական հոդված
@hy
2011年の論文
@ja
2011年論文
@yue
2011年論文
@zh-hant
2011年論文
@zh-hk
2011年論文
@zh-mo
2011年論文
@zh-tw
2011年论文
@wuu
name
Dysregulation of the ALS-assoc ...... eath and degeneration in mice.
@ast
Dysregulation of the ALS-assoc ...... eath and degeneration in mice.
@en
Dysregulation of the ALS-assoc ...... eath and degeneration in mice.
@nl
type
label
Dysregulation of the ALS-assoc ...... eath and degeneration in mice.
@ast
Dysregulation of the ALS-assoc ...... eath and degeneration in mice.
@en
Dysregulation of the ALS-assoc ...... eath and degeneration in mice.
@nl
prefLabel
Dysregulation of the ALS-assoc ...... eath and degeneration in mice.
@ast
Dysregulation of the ALS-assoc ...... eath and degeneration in mice.
@en
Dysregulation of the ALS-assoc ...... eath and degeneration in mice.
@nl
P2093
P2860
P356
P1476
Dysregulation of the ALS-assoc ...... eath and degeneration in mice.
@en
P2093
Alice Chen-Plotkin
Edward B Lee
Eric Swanson
Joe Malunda
Linda K Kwong
Lionel M Igaz
Matthew J Winton
Travis Unger
Virginia M-Y Lee
P2860
P304
P356
10.1172/JCI44867
P407
P577
2011-01-04T00:00:00Z