TDP-43-based animal models of neurodegeneration: new insights into ALS pathology and pathophysiology.
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TARDBP mutation analysis in TDP-43 proteinopathies and deciphering the toxicity of mutant TDP-43Exendin-4 ameliorates motor neuron degeneration in cellular and animal models of amyotrophic lateral sclerosisIdentification of genetic modifiers of TDP-43 neurotoxicity in DrosophilaThe ALS disease protein TDP-43 is actively transported in motor neuron axons and regulates axon outgrowth.Loss of ALS-associated TDP-43 in zebrafish causes muscle degeneration, vascular dysfunction, and reduced motor neuron axon outgrowth.New insights on NOX enzymes in the central nervous system.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.Early retinal neurodegeneration and impaired Ran-mediated nuclear import of TDP-43 in progranulin-deficient FTLDTargeting RNA binding proteins involved in neurodegeneration.Elevated mRNA-levels of distinct mitochondrial and plasma membrane Ca(2+) transporters in individual hypoglossal motor neurons of endstage SOD1 transgenic mice.Rodent models of TDP-43 proteinopathy: investigating the mechanisms of TDP-43-mediated neurodegenerationUnderstanding the role of TDP-43 and FUS/TLS in ALS and beyond.D-amino acid oxidase controls motoneuron degeneration through D-serine.Reactive oxygen species trigger motoneuron death in non-cell-autonomous models of ALS through activation of c-Abl signalingMutant induced pluripotent stem cell lines recapitulate aspects of TDP-43 proteinopathies and reveal cell-specific vulnerability.Slow development of ALS-like spinal cord pathology in mutant valosin-containing protein gene knock-in mice.Mechanisms of disease in frontotemporal lobar degeneration: gain of function versus loss of function effectsEndogenous TDP-43, but not FUS, contributes to stress granule assembly via G3BPLoss and gain of Drosophila TDP-43 impair synaptic efficacy and motor control leading to age-related neurodegeneration by loss-of-function phenotypesRodent models of TDP-43: recent advances.Tardbpl splicing rescues motor neuron and axonal development in a mutant tardbp zebrafish.RNA-binding proteins in neurodegenerative disease: TDP-43 and beyond.RNA-mediated toxicity in neurodegenerative diseaseGamma motor neurons survive and exacerbate alpha motor neuron degeneration in ALS.TDP-43 autoregulation: implications for disease.SOD1 and TDP-43 animal models of amyotrophic lateral sclerosis: recent advances in understanding disease toward the development of clinical treatments.TDP-43: the relationship between protein aggregation and neurodegeneration in amyotrophic lateral sclerosis and frontotemporal lobar degeneration.TDP-43 functions and pathogenic mechanisms implicated in TDP-43 proteinopathies.Molecular pathways of motor neuron injury in amyotrophic lateral sclerosis.Magnetic resonance imaging of pathological processes in rodent models of amyotrophic lateral sclerosis.Modeling human neurodegenerative diseases in transgenic systems.Fishing for causes and cures of motor neuron disorders.Physiological functions and pathobiology of TDP-43 and FUS/TLS proteins.A Quick Phenotypic Neurological Scoring System for Evaluating Disease Progression in the SOD1-G93A Mouse Model of ALS.Disease-associated mutations of TDP-43 promote turnover of the protein through the proteasomal pathway.TDP-43 regulates the microprocessor complex activity during in vitro neuronal differentiation.Clinical efficacy of edaravone for the treatment of amyotrophic lateral sclerosis.Autoregulation of TDP-43 mRNA levels involves interplay between transcription, splicing, and alternative polyA site selection.TDP-43 stabilises the processing intermediates of mitochondrial transcripts.Dioxins and related environmental contaminants increase TDP-43 levels.
P2860
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P2860
TDP-43-based animal models of neurodegeneration: new insights into ALS pathology and pathophysiology.
description
2010 nî lūn-bûn
@nan
2010 թուականի Դեկտեմբերին հրատարակուած գիտական յօդուած
@hyw
2010 թվականի դեկտեմբերին 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
TDP-43-based animal models of ...... pathology and pathophysiology.
@ast
TDP-43-based animal models of ...... pathology and pathophysiology.
@en
type
label
TDP-43-based animal models of ...... pathology and pathophysiology.
@ast
TDP-43-based animal models of ...... pathology and pathophysiology.
@en
prefLabel
TDP-43-based animal models of ...... pathology and pathophysiology.
@ast
TDP-43-based animal models of ...... pathology and pathophysiology.
@en
P2860
P356
P1476
TDP-43-based animal models of ...... pathology and pathophysiology.
@en
P2093
Iga Wegorzewska
Robert H Baloh
P2860
P304
P356
10.1159/000321547
P577
2010-12-03T00:00:00Z