Mutant TDP-43 in motor neurons promotes the onset and progression of ALS in rats.
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Pathogenic mutation of UBQLN2 impairs its interaction with UBXD8 and disrupts endoplasmic reticulum-associated protein degradationFrom animal models to human disease: a genetic approach for personalized medicine in ALSPathological mechanisms underlying TDP-43 driven neurodegeneration in FTLD-ALS spectrum disordersThe role of the immune system in neurodegenerative disorders: Adaptive or maladaptive?Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasisThe function of RNA-binding proteins at the synapse: implications for neurodegenerationOxr1 improves pathogenic cellular features of ALS-associated FUS and TDP-43 mutationsALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43.CDC7 inhibition blocks pathological TDP-43 phosphorylation and neurodegeneration.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 ratsCo-expression of truncated and full-length tau induces severe neurotoxicityMutant TDP-43 within motor neurons drives disease onset but not progression in amyotrophic lateral sclerosis.Motor neuron vulnerability and resistance in amyotrophic lateral sclerosis.Mitochondrial dysfunction and decrease in body weight of a transgenic knock-in mouse model for TDP-43.Pharmacological Modulation of Functional Phenotypes of Microglia in Neurodegenerative DiseasesProfiling 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.Nuclear TAR DNA-binding protein 43: A new target for amyotrophic lateral sclerosis treatmentCytoplasmic mislocalization of RNA splicing factors and aberrant neuronal gene splicing in TDP-43 transgenic pig brain.Rodent Models of Amyotrophic Lateral SclerosisGenome-wide approaches to dissect the roles of RNA binding proteins in translational control: implications for neurological diseasesEntorhinal cortical neurons are the primary targets of FUS mislocalization and ubiquitin aggregation in FUS transgenic rats.XBP1 depletion precedes ubiquitin aggregation and Golgi fragmentation in TDP-43 transgenic rats.TDP-43 in central nervous system development and function: clues to TDP-43-associated neurodegeneration.Pathogenic Ubqln2 gains toxic properties to induce neuron death.Reactive astrocytes secrete lcn2 to promote neuron death.Disease animal models of TDP-43 proteinopathy and their pre-clinical applications.Functional recovery in new mouse models of ALS/FTLD after clearance of pathological cytoplasmic TDP-43.The advent of AAV9 expands applications for brain and spinal cord gene delivery.Differential Roles of M1 and M2 Microglia in Neurodegenerative Diseases.Short-term suppression of A315T mutant human TDP-43 expression improves functional deficits in a novel inducible transgenic mouse model of FTLD-TDP and ALS.The role of endoplasmic reticulum stress in neurodegenerative disease.Rapamycin alleviates pathogenesis of a new Drosophila model of ALS-TDP.Increased Ubqln2 expression causes neuron death in transgenic ratsBiological Spectrum of Amyotrophic Lateral Sclerosis Prions.Temporal Expression of Mutant TDP-43 Correlates with Early Amyotrophic Lateral Sclerosis Phenotype and Motor Weakness.Increased cytoplasmic TDP-43 reduces global protein synthesis by interacting with RACK1 on polyribosomes.Overexpression of heat shock factor 1 maintains TAR DNA binding protein 43 solubility via induction of inducible heat shock protein 70 in cultured cells.Glycoprotein nonmetastatic melanoma protein B ameliorates skeletal muscle lesions in a SOD1G93A mouse model of amyotrophic lateral sclerosis.
P2860
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P2860
Mutant TDP-43 in motor neurons promotes the onset and progression of ALS in rats.
description
2011 nî lūn-bûn
@nan
2011年の論文
@ja
2011年論文
@yue
2011年論文
@zh-hant
2011年論文
@zh-hk
2011年論文
@zh-mo
2011年論文
@zh-tw
2011年论文
@wuu
2011年论文
@zh
2011年论文
@zh-cn
name
Mutant TDP-43 in motor neurons promotes the onset and progression of ALS in rats.
@ast
Mutant TDP-43 in motor neurons promotes the onset and progression of ALS in rats.
@en
type
label
Mutant TDP-43 in motor neurons promotes the onset and progression of ALS in rats.
@ast
Mutant TDP-43 in motor neurons promotes the onset and progression of ALS in rats.
@en
prefLabel
Mutant TDP-43 in motor neurons promotes the onset and progression of ALS in rats.
@ast
Mutant TDP-43 in motor neurons promotes the onset and progression of ALS in rats.
@en
P2093
P2860
P356
P1476
Mutant TDP-43 in motor neurons promotes the onset and progression of ALS in rats.
@en
P2093
Fangfang Bi
Hongxia Zhou
Jianbin Tong
Xu-Gang Xia
P2860
P304
P356
10.1172/JCI59130
P407
P577
2011-12-12T00:00:00Z