TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration.
about
Redox signalling directly regulates TDP-43 via cysteine oxidation and disulphide cross-linkingA 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 ALSMouse models of frontotemporal dementiaThe role of the immune system in neurodegenerative disorders: Adaptive or maladaptive?Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasisPotential mechanisms of progranulin-deficient FTLDThe function of RNA-binding proteins at the synapse: implications for neurodegenerationMicroglia centered pathogenesis in ALS: insights in cell interconnectivityDoes a loss of TDP-43 function cause neurodegeneration?ALS-associated TDP-43 induces endoplasmic reticulum stress, which drives cytoplasmic TDP-43 accumulation and stress granule formationCorticospinal Motor Neurons Are Susceptible to Increased ER Stress and Display Profound Degeneration in the Absence of UCHL1 FunctionRNA-processing protein TDP-43 regulates FOXO-dependent protein quality control in stress responseTDP-43-mediated neuron loss in vivo requires RNA-binding activityTDP-1/TDP-43 regulates stress signaling and age-dependent proteotoxicity in Caenorhabditis elegansThe bowel and beyond: the enteric nervous system in neurological disordersSystems biology of neurodegenerative diseasesThe complex molecular biology of amyotrophic lateral sclerosis (ALS)Identification of neuronal RNA targets of TDP-43-containing ribonucleoprotein complexesTar DNA binding protein-43 (TDP-43) associates with stress granules: analysis of cultured cells and pathological brain tissueExendin-4 ameliorates motor neuron degeneration in cellular and animal models of amyotrophic lateral sclerosisThe tau tubulin kinases TTBK1/2 promote accumulation of pathological TDP-43β-amyloid triggers ALS-associated TDP-43 pathology in AD modelsSelective frontoinsular von Economo neuron and fork cell loss in early behavioral variant frontotemporal dementia.TDP-43 transgenic mice develop spastic paralysis and neuronal inclusions characteristic of ALS and frontotemporal lobar degenerationExpansive gene transfer in the rat CNS rapidly produces amyotrophic lateral sclerosis relevant sequelae when TDP-43 is overexpressed.Phosphorylation promotes neurotoxicity in a Caenorhabditis elegans model of TDP-43 proteinopathy.Overexpression 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.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.Abnormal mitochondrial transport and morphology are common pathological denominators in SOD1 and TDP43 ALS mouse models.Protein kinase CK-1 inhibitors as new potential drugs for amyotrophic lateral sclerosis.Partial loss of TDP-43 function causes phenotypes of amyotrophic lateral sclerosis.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.Transgenic rat model of neurodegeneration caused by mutation in the TDP gene.
P2860
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P2860
TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration.
description
2009 nî lūn-bûn
@nan
2009 թուականի Հոկտեմբերին հրատարակուած գիտական յօդուած
@hyw
2009 թվականի հոտեմբերին հրատարակված գիտական հոդված
@hy
2009年の論文
@ja
2009年学术文章
@wuu
2009年学术文章
@zh-cn
2009年学术文章
@zh-hans
2009年学术文章
@zh-my
2009年学术文章
@zh-sg
2009年學術文章
@yue
name
TDP-43 mutant transgenic mice ...... totemporal lobar degeneration.
@ast
TDP-43 mutant transgenic mice ...... totemporal lobar degeneration.
@en
type
label
TDP-43 mutant transgenic mice ...... totemporal lobar degeneration.
@ast
TDP-43 mutant transgenic mice ...... totemporal lobar degeneration.
@en
prefLabel
TDP-43 mutant transgenic mice ...... totemporal lobar degeneration.
@ast
TDP-43 mutant transgenic mice ...... totemporal lobar degeneration.
@en
P2093
P2860
P356
P1476
TDP-43 mutant transgenic mice ...... totemporal lobar degeneration.
@en
P2093
Iga Wegorzewska
Nigel J Cairns
Robert H Baloh
Shaughn Bell
Timothy M Miller
P2860
P304
18809-18814
P356
10.1073/PNAS.0908767106
P407
P577
2009-10-15T00:00:00Z