The homozygote VCP(R¹⁵⁵H/R¹⁵⁵H) mouse model exhibits accelerated human VCP-associated disease pathology
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Mutations in the Human AAA+ Chaperone p97 and Related DiseasesA Fine Balance of Dietary Lipids Improves Pathology of a Murine Model of VCP-Associated Multisystem ProteinopathyMutations in valosin-containing protein (VCP) decrease ADP/ATP translocation across the mitochondrial membrane and impair energy metabolism in human neurons.Exercise training reverses skeletal muscle atrophy in an experimental model of VCP disease.Rapamycin and chloroquine: the in vitro and in vivo effects of autophagy-modifying drugs show promising results in valosin containing protein multisystem proteinopathy.Doa1 targets ubiquitinated substrates for mitochondria-associated degradation.Molecular regulation of autophagy and its implications for metabolic diseases.Neuronal-specific overexpression of a mutant valosin-containing protein associated with IBMPFD promotes aberrant ubiquitin and TDP-43 accumulation and cognitive dysfunction in transgenic mice.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."Get the Balance Right": Pathological Significance of Autophagy Perturbation in Neuromuscular DisordersValosin-containing protein (VCP/p97) inhibitors relieve Mitofusin-dependent mitochondrial defects due to VCP disease mutants.Paget's disease of bone-genetic and environmental factors.Different dynamic movements of wild-type and pathogenic VCPs and their cofactors to damaged mitochondria in a Parkin-mediated mitochondrial quality control system.VCP cooperates with UBXD1 to degrade mitochondrial outer membrane protein MCL1 in model of Huntington's disease.Activation of the NLRP3 Inflammasome Is Associated with Valosin-Containing Protein Myopathy.Dsc E3 ligase localization to the Golgi requires the ATPase Cdc48 and cofactor Ufd1 for activation of sterol regulatory element-binding protein in fission yeast.Loss of the novel Vcp (valosin containing protein) interactor Washc4 interferes with autophagy-mediated proteostasis in striated muscle and leads to myopathy in vivo
P2860
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P2860
The homozygote VCP(R¹⁵⁵H/R¹⁵⁵H) mouse model exhibits accelerated human VCP-associated disease pathology
description
2012 nî lūn-bûn
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2012 թուականին հրատարակուած գիտական յօդուած
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2012 թվականին հրատարակված գիտական հոդված
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2012年の論文
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2012年学术文章
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2012年学术文章
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2012年学术文章
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2012年学术文章
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2012年学术文章
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2012年學術文章
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name
The homozygote VCP(R¹⁵⁵H/R¹⁵⁵H ...... P-associated disease pathology
@ast
The homozygote VCP(R¹⁵⁵H/R¹⁵⁵H ...... P-associated disease pathology
@en
The homozygote VCP(R¹⁵⁵H/R¹⁵⁵H ...... P-associated disease pathology
@nl
type
label
The homozygote VCP(R¹⁵⁵H/R¹⁵⁵H ...... P-associated disease pathology
@ast
The homozygote VCP(R¹⁵⁵H/R¹⁵⁵H ...... P-associated disease pathology
@en
The homozygote VCP(R¹⁵⁵H/R¹⁵⁵H ...... P-associated disease pathology
@nl
prefLabel
The homozygote VCP(R¹⁵⁵H/R¹⁵⁵H ...... P-associated disease pathology
@ast
The homozygote VCP(R¹⁵⁵H/R¹⁵⁵H ...... P-associated disease pathology
@en
The homozygote VCP(R¹⁵⁵H/R¹⁵⁵H ...... P-associated disease pathology
@nl
P2093
P2860
P3181
P1433
P1476
The homozygote VCP(R¹⁵⁵H/R¹⁵⁵H ...... P-associated disease pathology
@en
P2093
Angèle Nalbandian
Christopher Nguyen
Giles Watts
Hong Z Yin
Jogeshwar Mukherjee
John Weiss
Katrina J Llewellyn
Mallikarjun Badadani
Masashi Kitazawa
Negar Khanlou
P2860
P304
P3181
P356
10.1371/JOURNAL.PONE.0046308
P407
P577
2012-01-01T00:00:00Z