Transgenic rat model of neurodegeneration caused by mutation in the TDP gene.
about
Pathogenic mutation of UBQLN2 impairs its interaction with UBXD8 and disrupts endoplasmic reticulum-associated protein degradationTARDBP mutation analysis in TDP-43 proteinopathies and deciphering the toxicity of mutant TDP-43From animal models to human disease: a genetic approach for personalized medicine in ALSALS Patient Stem Cells for Unveiling Disease Signatures of Motoneuron Susceptibility: Perspectives on the Deadly Mitochondria, ER Stress and Calcium TriadPharmacology of manipulating lean body massConverging mechanisms in ALS and FTD: disrupted RNA and protein homeostasisDoes a loss of TDP-43 function cause neurodegeneration?RNA-processing protein TDP-43 regulates FOXO-dependent protein quality control in stress responseTDP-43-mediated neuron loss in vivo requires RNA-binding activityFUS transgenic rats develop the phenotypes of amyotrophic lateral sclerosis and frontotemporal lobar degenerationTDP-43 Proteinopathy and ALS: Insights into Disease Mechanisms and Therapeutic Targets.The tau tubulin kinases TTBK1/2 promote accumulation of pathological TDP-43Expansive 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.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 ratsWild type human TDP-43 potentiates ALS-linked mutant TDP-43 driven progressive motor and cortical neuron degeneration with pathological features of ALS.RNA metabolism in neurodegenerative disease.The C-terminal TDP-43 fragments have a high aggregation propensity and harm neurons by a dominant-negative mechanismProfiling the genes affected by pathogenic TDP-43 in astrocytesDrosophila 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 dTDPThe influence of pathological mutations and proline substitutions in TDP-43 glycine-rich peptides on its amyloid properties and cellular toxicity.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.Neuroprotection for amyotrophic lateral sclerosis: role of stem cells, growth factors, and gene therapy.Genetic rodent models of amyotrophic lateral sclerosisTDP-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 signalingReversible behavioral phenotypes in a conditional mouse model of TDP-43 proteinopathiesTemporal expression of mutant LRRK2 in adult rats impairs dopamine reuptakeA fruitful endeavor: modeling ALS in the fruit flyRodent models of TDP-43 proteinopathy: investigating the mechanisms of TDP-43-mediated neurodegenerationModel systems of motor neuron diseases as a platform for studying pathogenic mechanisms and searching for therapeutic agents.TDP-43-based animal models of neurodegeneration: new insights into ALS pathology and pathophysiology.Expression of mutant TDP-43 induces neuronal dysfunction in transgenic mice.
P2860
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P2860
Transgenic rat model of neurodegeneration caused by mutation in the TDP gene.
description
2010 nî lūn-bûn
@nan
2010 թուականի Մարտին հրատարակուած գիտական յօդուած
@hyw
2010 թվականի մարտին 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
Transgenic rat model of neurodegeneration caused by mutation in the TDP gene.
@ast
Transgenic rat model of neurodegeneration caused by mutation in the TDP gene.
@en
Transgenic rat model of neurodegeneration caused by mutation in the TDP gene.
@nl
type
label
Transgenic rat model of neurodegeneration caused by mutation in the TDP gene.
@ast
Transgenic rat model of neurodegeneration caused by mutation in the TDP gene.
@en
Transgenic rat model of neurodegeneration caused by mutation in the TDP gene.
@nl
prefLabel
Transgenic rat model of neurodegeneration caused by mutation in the TDP gene.
@ast
Transgenic rat model of neurodegeneration caused by mutation in the TDP gene.
@en
Transgenic rat model of neurodegeneration caused by mutation in the TDP gene.
@nl
P2093
P2860
P1433
P1476
Transgenic rat model of neurodegeneration caused by mutation in the TDP gene
@en
P2093
Carlisle P Landel
Hongxia Zhou
Pedro Yuxing Xia
Robert Bowser
Xu Gang Xia
Yong-Jian Liu
P2860
P304
P356
10.1371/JOURNAL.PGEN.1000887
P50
P577
2010-03-26T00:00:00Z