Slow development of ALS-like spinal cord pathology in mutant valosin-containing protein gene knock-in mice.
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Pathogenic VCP mutations induce mitochondrial uncoupling and reduced ATP levelsOxidative stress and mitochondrial damage: importance in non-SOD1 ALSA Fine Balance of Dietary Lipids Improves Pathology of a Murine Model of VCP-Associated Multisystem Proteinopathyp97/VCP promotes degradation of CRBN substrate glutamine synthetase and neosubstrates.Deficient import of acetyl-CoA into the ER lumen causes neurodegeneration and propensity to infections, inflammation, and cancerProtein Quality Control and the Amyotrophic Lateral Sclerosis/Frontotemporal Dementia ContinuumMutations in valosin-containing protein (VCP) decrease ADP/ATP translocation across the mitochondrial membrane and impair energy metabolism in human neurons.Rapamycin and chloroquine: the in vitro and in vivo effects of autophagy-modifying drugs show promising results in valosin containing protein multisystem proteinopathy.Inhibitors of the AAA+ chaperone p97PET imaging studies show enhanced expression of mGluR5 and inflammatory response during progressive degeneration in ALS mouse model expressing SOD1-G93A geneDoa1 targets ubiquitinated substrates for mitochondria-associated degradation.VCP recruitment to mitochondria causes mitophagy impairment and neurodegeneration in models of Huntington's disease.Lipid-enriched diet rescues lethality and slows down progression in a murine model of VCP-associated disease.Amyotrophic lateral sclerosis: an update on recent genetic insights.FTD and ALS--translating mouse studies into clinical trials.Targeting mitochondrial metal dyshomeostasis for the treatment of neurodegeneration.ALS/FTLD: experimental models and reality.Different dynamic movements of wild-type and pathogenic VCPs and their cofactors to damaged mitochondria in a Parkin-mediated mitochondrial quality control system.Mutation of the caspase-3 cleavage site in the astroglial glutamate transporter EAAT2 delays disease progression and extends lifespan in the SOD1-G93A mouse model of ALS.
P2860
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P2860
Slow development of ALS-like spinal cord pathology in mutant valosin-containing protein gene knock-in mice.
description
2012 nî lūn-bûn
@nan
2012年の論文
@ja
2012年学术文章
@wuu
2012年学术文章
@zh-cn
2012年学术文章
@zh-hans
2012年学术文章
@zh-my
2012年学术文章
@zh-sg
2012年學術文章
@yue
2012年學術文章
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2012年學術文章
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name
Slow development of ALS-like s ...... ng protein gene knock-in mice.
@ast
Slow development of ALS-like s ...... ng protein gene knock-in mice.
@en
type
label
Slow development of ALS-like s ...... ng protein gene knock-in mice.
@ast
Slow development of ALS-like s ...... ng protein gene knock-in mice.
@en
prefLabel
Slow development of ALS-like s ...... ng protein gene knock-in mice.
@ast
Slow development of ALS-like s ...... ng protein gene knock-in mice.
@en
P2093
P2860
P356
P1476
Slow development of ALS-like s ...... ng protein gene knock-in mice.
@en
P2093
A Nalbandian
K J Llewellyn
T Mozaffar
V E Kimonis
P2860
P356
10.1038/CDDIS.2012.115
P577
2012-08-16T00:00:00Z