Nuclear localization of human SOD1 and mutant SOD1-specific disruption of survival motor neuron protein complex in transgenic amyotrophic lateral sclerosis mice
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The Cu, Zn Superoxide Dismutase: Not Only a Dismutase EnzymeDiverse expression of selected SMN complex proteins in humans with sporadic amyotrophic lateral sclerosis and in a transgenic rat model of familial form of the disease.GNX-4728, a novel small molecule drug inhibitor of mitochondrial permeability transition, is therapeutic in a mouse model of amyotrophic lateral sclerosis.ALS-causative mutations in FUS/TLS confer gain and loss of function by altered association with SMN and U1-snRNP.Mutant superoxide dismutase 1 (SOD1), a cause of amyotrophic lateral sclerosis, disrupts the recruitment of SMN, the spinal muscular atrophy protein to nuclear Cajal bodies.FUS-SMN protein interactions link the motor neuron diseases ALS and SMAMitochondrial DNMT3A and DNA methylation in skeletal muscle and CNS of transgenic mouse models of ALS.Overexpression of survival motor neuron improves neuromuscular function and motor neuron survival in mutant SOD1 mice.Species-dependent neuropathology in transgenic SOD1 pigs.Protein aggregation in amyotrophic lateral sclerosis.Gem depletion: amyotrophic lateral sclerosis and spinal muscular atrophy crossover.Cajal bodies in neuronsALS-associated mutations in FUS disrupt the axonal distribution and function of SMN.Enhancing survival motor neuron expression extends lifespan and attenuates neurodegeneration in mutant TDP-43 mice.Motor Neuron Gene Therapy: Lessons from Spinal Muscular Atrophy for Amyotrophic Lateral Sclerosis.
P2860
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P2860
Nuclear localization of human SOD1 and mutant SOD1-specific disruption of survival motor neuron protein complex in transgenic amyotrophic lateral sclerosis mice
description
2012 nî lūn-bûn
@nan
2012年の論文
@ja
2012年論文
@yue
2012年論文
@zh-hant
2012年論文
@zh-hk
2012年論文
@zh-mo
2012年論文
@zh-tw
2012年论文
@wuu
2012年论文
@zh
2012年论文
@zh-cn
name
Nuclear localization of human ...... trophic lateral sclerosis mice
@ast
Nuclear localization of human ...... trophic lateral sclerosis mice
@en
type
label
Nuclear localization of human ...... trophic lateral sclerosis mice
@ast
Nuclear localization of human ...... trophic lateral sclerosis mice
@en
prefLabel
Nuclear localization of human ...... trophic lateral sclerosis mice
@ast
Nuclear localization of human ...... trophic lateral sclerosis mice
@en
P2093
P2860
P1476
Nuclear localization of human ...... trophic lateral sclerosis mice
@en
P2093
Barry Gertz
Lee J Martin
Margaret Wong
P2860
P304
P356
10.1097/NEN.0B013E318244B635
P577
2012-02-01T00:00:00Z