Wild-type human TDP-43 expression causes TDP-43 phosphorylation, mitochondrial aggregation, motor deficits, and early mortality in transgenic mice.
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VAPB interacts with the mitochondrial protein PTPIP51 to regulate calcium homeostasisTARDBP 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 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 vivoALS Patient Stem Cells for Unveiling Disease Signatures of Motoneuron Susceptibility: Perspectives on the Deadly Mitochondria, ER Stress and Calcium TriadTDP-43: a new player on the AD field?Pathological mechanisms underlying TDP-43 driven neurodegeneration in FTLD-ALS spectrum disordersMouse models of frontotemporal dementiaThe role of the immune system in neurodegenerative disorders: Adaptive or maladaptive?Mitochondrial dysfunction in amyotrophic lateral sclerosis - a valid pharmacological target?Oxidative stress and mitochondrial damage: importance in non-SOD1 ALSDoes a loss of TDP-43 function cause neurodegeneration?RNA-processing protein TDP-43 regulates FOXO-dependent protein quality control in stress responseTDP-1/TDP-43 regulates stress signaling and age-dependent proteotoxicity in Caenorhabditis elegansEmerging mechanisms of molecular pathology in ALSOxr1 improves pathogenic cellular features of ALS-associated FUS and TDP-43 mutationsFUS Interacts with HSP60 to Promote Mitochondrial DamageAberrant localization of FUS and TDP43 is associated with misfolding of SOD1 in amyotrophic lateral sclerosisGait disturbances in dystrophic hamsters.Non-human primate model of amyotrophic lateral sclerosis with cytoplasmic mislocalization of TDP-43Overexpression of human wild-type FUS causes progressive motor neuron degeneration in an age- and dose-dependent fashion.ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43.Overexpression of ALS-associated p.M337V human TDP-43 in mice worsens disease features compared to wild-type human TDP-43 mice.Abnormal mitochondrial transport and morphology are common pathological denominators in SOD1 and TDP43 ALS mouse models.Expression 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.Motor neuron intrinsic and extrinsic mechanisms contribute to the pathogenesis of FUS-associated amyotrophic lateral sclerosis.Loss of function CHCHD10 mutations in cytoplasmic TDP-43 accumulation and synaptic integrityAstrocytic TDP-43 pathology in Alexander diseaseMitochondrial dysfunction and decrease in body weight of a transgenic knock-in mouse model for TDP-43.TDP-43 suppresses tau expression via promoting its mRNA instability.RNA metabolism in neurodegenerative disease.TDP-43 regulates its mRNA levels through a negative feedback loop.The C-terminal TDP-43 fragments have a high aggregation propensity and harm neurons by a dominant-negative mechanismAbnormalities 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.The aggregation and neurotoxicity of TDP-43 and its ALS-associated 25 kDa fragment are differentially affected by molecular chaperones in Drosophila.Genetic rodent models of amyotrophic lateral sclerosis
P2860
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P2860
Wild-type human TDP-43 expression causes TDP-43 phosphorylation, mitochondrial aggregation, motor deficits, and early mortality in transgenic mice.
description
2010 nî lūn-bûn
@nan
2010 թուականի Օգոստոսին հրատարակուած գիտական յօդուած
@hyw
2010 թվականի օգոստոսին հրատարակված գիտական հոդված
@hy
2010年の論文
@ja
2010年論文
@yue
2010年論文
@zh-hant
2010年論文
@zh-hk
2010年論文
@zh-mo
2010年論文
@zh-tw
2010年论文
@wuu
name
Wild-type human TDP-43 express ...... mortality in transgenic mice.
@ast
Wild-type human TDP-43 express ...... mortality in transgenic mice.
@en
Wild-type human TDP-43 express ...... mortality in transgenic mice.
@nl
type
label
Wild-type human TDP-43 express ...... mortality in transgenic mice.
@ast
Wild-type human TDP-43 express ...... mortality in transgenic mice.
@en
Wild-type human TDP-43 express ...... mortality in transgenic mice.
@nl
prefLabel
Wild-type human TDP-43 express ...... mortality in transgenic mice.
@ast
Wild-type human TDP-43 express ...... mortality in transgenic mice.
@en
Wild-type human TDP-43 express ...... mortality in transgenic mice.
@nl
P2093
P2860
P50
P1476
Wild-type human TDP-43 express ...... mortality in transgenic mice.
@en
P2093
Eileen McGowan
Hong Sheng
Jada Lewis
Jimei Tong
Joshua Knight
Kevin Boylan
Mike Hutton
Monica Castanedes Casey
Simon D'Alton
Tania F Gendron
P2860
P304
10851-10859
P356
10.1523/JNEUROSCI.1630-10.2010
P407
P577
2010-08-01T00:00:00Z