VCP disease associated with myopathy, Paget disease of bone and frontotemporal dementia: review of a unique disorder.
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Imbalances in p97 co-factor interactions in human proteinopathyExome sequencing reveals VCP mutations as a cause of familial ALSPhenotypic Heterogeneity of Monogenic Frontotemporal DementiaCardiac-Restricted Expression of VCP/TER94 RNAi or Disease Alleles Perturbs Drosophila Heart Structure and Impairs Function.The homozygote VCP(R¹⁵⁵H/R¹⁵⁵H) mouse model exhibits accelerated human VCP-associated disease pathologyInactivation of VCP/ter94 suppresses retinal pathology caused by misfolded rhodopsin in DrosophilaMutations in the Human AAA+ Chaperone p97 and Related DiseasesVCP/p97 is essential for maturation of ubiquitin-containing autophagosomes and this function is impaired by mutations that cause IBMPFDRare Manifestation of a c.290 C>T, p.Gly97Glu VCP MutationHereditary inclusion body myopathy-linked p97/VCP mutations in the NH2 domain and the D1 ring modulate p97/VCP ATPase activity and D2 ring conformationThe p97/VCP ATPase is critical in muscle atrophy and the accelerated degradation of muscle proteinsA Fine Balance of Dietary Lipids Improves Pathology of a Murine Model of VCP-Associated Multisystem ProteinopathyThe Myoblast C2C12 Transfected with Mutant Valosin-Containing Protein Exhibits Delayed Stress Granule Resolution on Oxidative Stress.METTL21C is a potential pleiotropic gene for osteoporosis and sarcopenia acting through the modulation of the NF-κB signaling pathway.Protein Quality Control and the Amyotrophic Lateral Sclerosis/Frontotemporal Dementia ContinuumVCP associated inclusion body myopathy and paget disease of bone knock-in mouse model exhibits tissue pathology typical of human disease.Myogenic differentiation of VCP disease-induced pluripotent stem cells: A novel platform for drug discoveryPathogenic VCP/TER94 alleles are dominant actives and contribute to neurodegeneration by altering cellular ATP level in a Drosophila IBMPFD modelA Brazilian family with hereditary inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia.Inclusion body myopathy, Paget's disease of the bone and fronto-temporal dementia: a disorder of autophagyTDP-43 mediates degeneration in a novel Drosophila model of disease caused by mutations in VCP/p97.Valosin-containing protein (VCP) in novel feedback machinery between abnormal protein accumulation and transcriptional suppressionAssembly, structure, and function of the 26S proteasome.Rare mutations in SQSTM1 modify susceptibility to frontotemporal lobar degenerationThe VCP/p97 system at a glance: connecting cellular function to disease pathogenesis.Clinical phenotypes and genetic biomarkers of FTLD.The emerging roles of microRNAs in the pathogenesis of frontotemporal dementia-amyotrophic lateral sclerosis (FTD-ALS) spectrum disordersClinical, neuroimaging and neuropathological features of a new chromosome 9p-linked FTD-ALS family.Valosin-containing protein gene mutations: cellular phenotypes relevant to neurodegeneration.Immunoreactivity of valosin-containing protein in sporadic amyotrophic lateral sclerosis and in a case of its novel mutantExercise training reverses skeletal muscle atrophy in an experimental model of VCP disease.Global gene expression profiling in R155H knock-in murine model of VCP disease.Targeted excision of VCP R155H mutation by Cre-LoxP technology as a promising therapeutic strategy for valosin-containing protein disease.Targeted sequencing and identification of genetic variants in sporadic inclusion body myositis.Rapamycin and chloroquine: the in vitro and in vivo effects of autophagy-modifying drugs show promising results in valosin containing protein multisystem proteinopathy.Global gene profiling of VCP-associated inclusion body myopathy.Valosin-containing protein (VCP) mutations in sporadic amyotrophic lateral sclerosis.IBMPFD Disease-Causing Mutant VCP/p97 Proteins Are Targets of Autophagic-Lysosomal Degradation.The molecular basis of the frontotemporal lobar degeneration-amyotrophic lateral sclerosis spectrumA progressive translational mouse model of human valosin-containing protein disease: the VCP(R155H/+) mouse
P2860
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P2860
VCP disease associated with myopathy, Paget disease of bone and frontotemporal dementia: review of a unique disorder.
description
2008 nî lūn-bûn
@nan
2008 թուականի Սեպտեմբերին հրատարակուած գիտական յօդուած
@hyw
2008 թվականի սեպտեմբերին հրատարակված գիտական հոդված
@hy
2008年の論文
@ja
2008年論文
@yue
2008年論文
@zh-hant
2008年論文
@zh-hk
2008年論文
@zh-mo
2008年論文
@zh-tw
2008年论文
@wuu
name
VCP disease associated with my ...... : review of a unique disorder.
@ast
VCP disease associated with my ...... : review of a unique disorder.
@en
VCP disease associated with my ...... : review of a unique disorder.
@nl
type
label
VCP disease associated with my ...... : review of a unique disorder.
@ast
VCP disease associated with my ...... : review of a unique disorder.
@en
VCP disease associated with my ...... : review of a unique disorder.
@nl
prefLabel
VCP disease associated with my ...... : review of a unique disorder.
@ast
VCP disease associated with my ...... : review of a unique disorder.
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VCP disease associated with my ...... : review of a unique disorder.
@nl
P2093
P1476
VCP disease associated with my ...... : review of a unique disorder.
@en
P2093
Erin Fulchiero
Giles Watts
Jouni Vesa
Virginia E Kimonis
P304
P356
10.1016/J.BBADIS.2008.09.003
P407
P577
2008-09-18T00:00:00Z