Transgenic mice expressing mutant forms VCP/p97 recapitulate the full spectrum of IBMPFD including degeneration in muscle, brain and bone. - Wikidata - wikimedia - TriplyDB

Transgenic mice expressing mutant forms VCP/p97 recapitulate the full spectrum of IBMPFD including degeneration in muscle, brain and bone.

Transgenic mice expressing mutant forms VCP/p97 recapitulate the full spectrum of IBMPFD including degeneration in muscle, brain and bone.

http://www.wikidata.org/entity/Q48312639

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Pathogenic VCP mutations induce mitochondrial uncoupling and reduced ATP levels Exome sequencing reveals VCP mutations as a cause of familial ALS Mouse models of frontotemporal dementia The homozygote VCP(R¹⁵⁵H/R¹⁵⁵H) mouse model exhibits accelerated human VCP-associated disease pathology Targeting protein homeostasis in sporadic inclusion body myositis.Disrupted autophagy undermines skeletal muscle adaptation and integrity Mutations in the Human AAA+ Chaperone p97 and Related Diseases Tar DNA binding protein-43 (TDP-43) associates with stress granules: analysis of cultured cells and pathological brain tissue 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.Protein Homeostasis in Amyotrophic Lateral Sclerosis: Therapeutic Opportunities?Tumor Suppressor RARRES1 Regulates DLG2, PP2A, VCP, EB1, and Ankrd26.VCP associated inclusion body myopathy and paget disease of bone knock-in mouse model exhibits tissue pathology typical of human disease.Cytokine profiling in patients with VCP-associated disease.Inclusion body myopathy, Paget's disease of the bone and fronto-temporal dementia: a disorder of autophagy Getting folded: chaperone proteins in muscle development, maintenance and disease.A novel conserved isoform of the ubiquitin ligase UFD2a/UBE4B is expressed exclusively in mature striated muscle cells.Liver cytochrome P450 3A ubiquitination in vivo by gp78/autocrine motility factor receptor and C terminus of Hsp70-interacting protein (CHIP) E3 ubiquitin ligases: physiological and pharmacological relevance.p97-containing complexes in proliferation control and cancer: emerging culprits or guilt by association?Sortilin-mediated endocytosis determines levels of the frontotemporal dementia protein, progranulin.Contributions of the measles virus nucleocapsid gene and the SQSTM1/p62(P392L) mutation to Paget's disease.Liver cytochrome P450 3A endoplasmic reticulum-associated degradation: a major role for the p97 AAA ATPase in cytochrome P450 3A extraction into the cytosol.The complexities of p97 function in health and disease Valosin-containing protein gene mutations: cellular phenotypes relevant to neurodegeneration.A network of RNA and protein interactions in Fronto Temporal Dementia.Mutant p62P392L stimulation of osteoclast differentiation in Paget's disease of bone Valosin-containing protein and neurofibromin interact to regulate dendritic spine density Autophagy in dementias.Valosin containing protein associated fronto-temporal lobar degeneration: clinical presentation, pathologic features and pathogenesis.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.The genetic pleiotropy of musculoskeletal aging.Slow development of ALS-like spinal cord pathology in mutant valosin-containing protein gene knock-in mice.Retrotransposon activation contributes to neurodegeneration in a Drosophila TDP-43 model of ALS A progressive translational mouse model of human valosin-containing protein disease: the VCP(R155H/+) mouse Structural and functional deviations in disease-associated p97 mutants.Doa1 targets ubiquitinated substrates for mitochondria-associated degradation.

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Transgenic mice expressing mutant forms VCP/p97 recapitulate the full spectrum of IBMPFD including degeneration in muscle, brain and bone.

http://www.wikidata.org/entity/Q48312639

description

2010 nî lūn-bûn

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2010年の論文

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2010年学术文章

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2010年学术文章

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2010年学术文章

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2010年学术文章

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2010年学术文章

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2010年学术文章

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2010年學術文章

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2010年學術文章

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name

Transgenic mice expressing mut ...... ion in muscle, brain and bone.

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Transgenic mice expressing mut ...... ion in muscle, brain and bone.

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type

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Transgenic mice expressing mut ...... ion in muscle, brain and bone.

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Transgenic mice expressing mut ...... ion in muscle, brain and bone.

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Transgenic mice expressing mut ...... ion in muscle, brain and bone.

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Transgenic mice expressing mut ...... ion in muscle, brain and bone.

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Human Molecular Genetics

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Transgenic mice expressing mut ...... ion in muscle, brain and bone.

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Alexander C Wright

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Hongbo Lu

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J Paul Taylor

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Sara K Custer

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P2860

Involvement of valosin-containing protein, an ATPase Co-purified with IkappaBalpha and 26 S proteasome, in ubiquitin-proteasome-mediated degradation of IkappaBalpha

Sarcoplasmic redistribution of nuclear TDP-43 in inclusion body myositis

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

Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis

beta-TrCP mediates the signal-induced ubiquitination of IkappaBbeta

VCP (p97) regulates NFkappaB signaling pathway, which is important for metastasis of osteosarcoma cell line

Nucleotide dependent motion and mechanism of action of p97/VCP

Hereditary inclusion body myopathy-linked p97/VCP mutations in the NH2 domain and the D1 ring modulate p97/VCP ATPase activity and D2 ring conformation

Valosin-containing protein and the pathogenesis of frontotemporal dementia associated with inclusion body myopathy

Inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia is caused by mutant valosin-containing protein

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1741-1755

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9

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19

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Manuela Neumann

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41421705

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20147319

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