VCP associated inclusion body myopathy and paget disease of bone knock-in mouse model exhibits tissue pathology typical of human disease.
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Pathogenic VCP mutations induce mitochondrial uncoupling and reduced ATP levelsMouse models of frontotemporal dementiaThe homozygote VCP(R¹⁵⁵H/R¹⁵⁵H) mouse model exhibits accelerated human VCP-associated disease pathologyThe Cdc48 protein and its cofactor Vms1 are involved in Cdc13 protein degradation.Mutations in the Human AAA+ Chaperone p97 and Related DiseasesMitochondrial protein quality control: the mechanisms guarding mitochondrial healthA Fine Balance of Dietary Lipids Improves Pathology of a Murine Model of VCP-Associated Multisystem ProteinopathyBoning up on autophagy: the role of autophagy in skeletal biologyThe Myoblast C2C12 Transfected with Mutant Valosin-Containing Protein Exhibits Delayed Stress Granule Resolution on Oxidative Stress.VCP is essential for mitochondrial quality control by PINK1/Parkin and this function is impaired by VCP mutations.Ubiquilin-2 drives NF-κB activity and cytosolic TDP-43 aggregation in neuronal cells.p97/VCP promotes degradation of CRBN substrate glutamine synthetase and neosubstrates.Withania somnifera Reverses Transactive Response DNA Binding Protein 43 Proteinopathy in a Mouse Model of Amyotrophic Lateral Sclerosis/Frontotemporal Lobar Degeneration.Myogenic differentiation of VCP disease-induced pluripotent stem cells: A novel platform for drug discoveryThe VCP/p97 system at a glance: connecting cellular function to disease pathogenesis.Derlin-1 regulates mutant VCP-linked pathogenesis and endoplasmic reticulum stress-induced apoptosis.Heteromeric p97/p97R155C complexes induce dominant negative changes in wild-type and autophagy 9-deficient Dictyostelium strainsNucleocytoplasmic shuttling of valosin-containing protein (VCP/p97) regulated by its N domain and C-terminal region.Exercise 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.Rapamycin and chloroquine: the in vitro and in vivo effects of autophagy-modifying drugs show promising results in valosin containing protein multisystem proteinopathy.Valosin-containing protein and neurofibromin interact to regulate dendritic spine densityAutophagy in dementias.A functional deficiency of TERA/VCP/p97 contributes to impaired DNA repair in multiple polyglutamine diseasesGlobal gene profiling of VCP-associated inclusion body myopathy.New mouse models for metabolic bone diseases generated by genome-wide ENU mutagenesis.Slow development of ALS-like spinal cord pathology in mutant valosin-containing protein gene knock-in mice.A progressive translational mouse model of human valosin-containing protein disease: the VCP(R155H/+) mouseStructural and functional deviations in disease-associated p97 mutants.mTOR dysfunction contributes to vacuolar pathology and weakness in valosin-containing protein associated inclusion body myopathy.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.Lipid-enriched diet rescues lethality and slows down progression in a murine model of VCP-associated disease.The multiple faces of valosin-containing protein-associated diseases: inclusion body myopathy with Paget's disease of bone, frontotemporal dementia, and amyotrophic lateral sclerosis.Pathogenesis of Paget disease of bone.Amyotrophic lateral sclerosis: an update on recent genetic insights.Emerging roles for hnRNPs in post-transcriptional regulation: what can we learn from flies?Deregulation of TDP-43 in amyotrophic lateral sclerosis triggers nuclear factor κB-mediated pathogenic pathwaysFTD and ALS--translating mouse studies into clinical trials.Paget's disease of bone-genetic and environmental factors.
P2860
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P2860
VCP associated inclusion body myopathy and paget disease of bone knock-in mouse model exhibits tissue pathology typical of human disease.
description
2010 nî lūn-bûn
@nan
2010 թուականի Հոկտեմբերին հրատարակուած գիտական յօդուած
@hyw
2010 թվականի հոտեմբերին հրատարակված գիտական հոդված
@hy
2010年の論文
@ja
2010年論文
@yue
2010年論文
@zh-hant
2010年論文
@zh-hk
2010年論文
@zh-mo
2010年論文
@zh-tw
2010年论文
@wuu
name
VCP associated inclusion body ...... logy typical of human disease.
@ast
VCP associated inclusion body ...... logy typical of human disease.
@en
type
label
VCP associated inclusion body ...... logy typical of human disease.
@ast
VCP associated inclusion body ...... logy typical of human disease.
@en
prefLabel
VCP associated inclusion body ...... logy typical of human disease.
@ast
VCP associated inclusion body ...... logy typical of human disease.
@en
P2093
P2860
P1433
P1476
VCP associated inclusion body ...... logy typical of human disease.
@en
P2093
Angèle Nalbandian
Douglas C Wallace
Giles D Watts
Hailing Su
Jasmin Tanaja
Jogeshwar Mukherjee
Jouni Vesa
Mallikarjun Badadani
Masashi Kitazawa
P2860
P356
10.1371/JOURNAL.PONE.0013183
P407
P577
2010-10-05T00:00:00Z