VCP associated inclusion body myopathy and paget disease of bone knock-in mouse model exhibits tissue pathology typical of human disease. - Wikidata - awgldk - TriplyDB

VCP associated inclusion body myopathy and paget disease of bone knock-in mouse model exhibits tissue pathology typical of human disease.

VCP associated inclusion body myopathy and paget disease of bone knock-in mouse model exhibits tissue pathology typical of human disease.

http://www.wikidata.org/entity/Q33721995

about

Pathogenic VCP mutations induce mitochondrial uncoupling and reduced ATP levels Mouse models of frontotemporal dementia The homozygote VCP(R¹⁵⁵H/R¹⁵⁵H) mouse model exhibits accelerated human VCP-associated disease pathology The Cdc48 protein and its cofactor Vms1 are involved in Cdc13 protein degradation.Mutations in the Human AAA+ Chaperone p97 and Related Diseases Mitochondrial protein quality control: the mechanisms guarding mitochondrial health A Fine Balance of Dietary Lipids Improves Pathology of a Murine Model of VCP-Associated Multisystem Proteinopathy Boning up on autophagy: the role of autophagy in skeletal biology The 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 discovery The 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 strains Nucleocytoplasmic 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 density Autophagy in dementias.A functional deficiency of TERA/VCP/p97 contributes to impaired DNA repair in multiple polyglutamine diseases Global 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/+) mouse Structural 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 pathways FTD and ALS--translating mouse studies into clinical trials.Paget's disease of bone-genetic and environmental factors.

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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.

http://www.wikidata.org/entity/Q33721995

description

2010 nî lūn-bûn

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2010 թուականի Հոկտեմբերին հրատարակուած գիտական յօդուած

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2010 թվականի հոտեմբերին հրատարակված գիտական հոդված

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2010年の論文

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2010年論文

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2010年論文

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2010年論文

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2010年論文

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2010年論文

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2010年论文

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VCP associated inclusion body ...... logy typical of human disease.

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VCP associated inclusion body ...... logy typical of human disease.

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VCP associated inclusion body ...... logy typical of human disease.

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JOURNAL.PONE.0013183

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VCP associated inclusion body ...... logy typical of human disease.

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Angèle Nalbandian

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Douglas C Wallace

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Eric Dec

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Giles D Watts

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Hailing Su

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Jasmin Tanaja

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Jogeshwar Mukherjee

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Jouni Vesa

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Mallikarjun Badadani

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Masashi Kitazawa

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P2860

Valosin-containing protein is a multi-ubiquitin chain-targeting factor required in ubiquitin-proteasome degradation

A complex of mammalian ufd1 and npl4 links the AAA-ATPase, p97, to ubiquitin and nuclear transport pathways

Mobilization of processed, membrane-tethered SPT23 transcription factor by CDC48(UFD1/NPL4), a ubiquitin-selective chaperone.

The AAA ATPase Cdc48/p97 and its partners transport proteins from the ER into the cytosol.

AAA-ATPase p97/Cdc48p, a cytosolic chaperone required for endoplasmic reticulum-associated protein degradation.

VCP/p97 is essential for maturation of ubiquitin-containing autophagosomes and this function is impaired by mutations that cause IBMPFD

Functional ATPase activity of p97/valosin-containing protein (VCP) is required for the quality control of endoplasmic reticulum in neuronally differentiated mammalian PC12 cells

AAA+ superfamily ATPases: common structure--diverse function

Clinical delineation and localization to chromosome 9p13.3-p12 of a unique dominant disorder in four families: hereditary inclusion body myopathy, Paget disease of bone, and frontotemporal dementia

p47 is a cofactor for p97-mediated membrane fusion

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scholarly article

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10.1371/JOURNAL.PONE.0013183

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