TDP-43, a neuro-pathosignature factor, is essential for early mouse embryogenesis.
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TARDBP mutation analysis in TDP-43 proteinopathies and deciphering the toxicity of mutant TDP-43Pathological mechanisms underlying TDP-43 driven neurodegeneration in FTLD-ALS spectrum disordersMouse models of frontotemporal dementiaConverging mechanisms in ALS and FTD: disrupted RNA and protein homeostasisPotential mechanisms of progranulin-deficient FTLDTherapeutic and diagnostic challenges for frontotemporal dementiaDoes a loss of TDP-43 function cause neurodegeneration?The neurogenetics of alternative splicingTDP-43 Proteinopathy and ALS: Insights into Disease Mechanisms and Therapeutic Targets.Akirin2 is essential for the formation of the cerebral cortexIdentification of neuronal RNA targets of TDP-43-containing ribonucleoprotein complexesTDP-43 is a transcriptional repressor: the testis-specific mouse acrv1 gene is a TDP-43 target in vivo.TDP-43 transgenic mice develop spastic paralysis and neuronal inclusions characteristic of ALS and frontotemporal lobar degenerationLoss of murine TDP-43 disrupts motor function and plays an essential role in embryogenesis.Expansive gene transfer in the rat CNS rapidly produces amyotrophic lateral sclerosis relevant sequelae when TDP-43 is overexpressed.The ALS disease protein TDP-43 is actively transported in motor neuron axons and regulates axon outgrowth.ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43.Loss of ALS-associated TDP-43 in zebrafish causes muscle degeneration, vascular dysfunction, and reduced motor neuron axon outgrowth.Partial loss of TDP-43 function causes phenotypes of amyotrophic lateral sclerosis.Wild type human TDP-43 potentiates ALS-linked mutant TDP-43 driven progressive motor and cortical neuron degeneration with pathological features of ALS.TDP-43 is a developmentally regulated protein essential for early embryonic development.Mitochondrial dysfunction and decrease in body weight of a transgenic knock-in mouse model for TDP-43.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 mechanismDrosophila 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.Neuronal function and dysfunction of Drosophila dTDPElevated expression of TDP-43 in the forebrain of mice is sufficient to cause neurological and pathological phenotypes mimicking FTLD-U.Interaction with polyglutamine aggregates reveals a Q/N-rich domain in TDP-43.Nuclear TAR DNA-binding protein 43: A new target for amyotrophic lateral sclerosis treatmentAltered distributions of Gemini of coiled bodies and mitochondria in motor neurons of TDP-43 transgenic mice.Deletion of TDP-43 down-regulates Tbc1d1, a gene linked to obesity, and alters body fat metabolism.Dysregulation of the ALS-associated gene TDP-43 leads to neuronal death and degeneration in mice.TDP-43 in aging and Alzheimer's disease - a reviewTranscription factors expressed in olfactory bulb local progenitor cells revealed by genome-wide transcriptome profiling.TDP-43 potentiates alpha-synuclein toxicity to dopaminergic neurons in transgenic miceTDP-43 neurotoxicity and protein aggregation modulated by heat shock factor and insulin/IGF-1 signalingThe pathological phenotypes of human TDP-43 transgenic mouse models are independent of downregulation of mouse Tdp-43Downregulation of microRNA-9 in iPSC-derived neurons of FTD/ALS patients with TDP-43 mutations.Allele-specific knockdown of ALS-associated mutant TDP-43 in neural stem cells derived from induced pluripotent stem cellsGene targeting of mouse Tardbp negatively affects Masp2 expression.
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P2860
TDP-43, a neuro-pathosignature factor, is essential for early mouse embryogenesis.
description
2010 nî lūn-bûn
@nan
2010年の論文
@ja
2010年学术文章
@wuu
2010年学术文章
@zh
2010年学术文章
@zh-cn
2010年学术文章
@zh-hans
2010年学术文章
@zh-my
2010年学术文章
@zh-sg
2010年學術文章
@yue
2010年學術文章
@zh-hant
name
TDP-43, a neuro-pathosignature factor, is essential for early mouse embryogenesis.
@en
TDP-43, a neuro-pathosignature factor, is essential for early mouse embryogenesis.
@nl
type
label
TDP-43, a neuro-pathosignature factor, is essential for early mouse embryogenesis.
@en
TDP-43, a neuro-pathosignature factor, is essential for early mouse embryogenesis.
@nl
prefLabel
TDP-43, a neuro-pathosignature factor, is essential for early mouse embryogenesis.
@en
TDP-43, a neuro-pathosignature factor, is essential for early mouse embryogenesis.
@nl
P2093
P356
P1433
P1476
TDP-43, a neuro-pathosignature factor, is essential for early mouse embryogenesis.
@en
P2093
C-K James Shen
Lien-Szu Wu
Shin-Chen Hou
Wei-Cheng Cheng
Yu-Ting Yan
P356
10.1002/DVG.20584
P50
P577
2010-01-01T00:00:00Z