The ubiquitin-selective chaperone CDC-48/p97 links myosin assembly to human myopathy. - Wikidata - awgldk - TriplyDB

The ubiquitin-selective chaperone CDC-48/p97 links myosin assembly to human myopathy.

The ubiquitin-selective chaperone CDC-48/p97 links myosin assembly to human myopathy.

http://www.wikidata.org/entity/Q40157250

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Structural and functional characterization of the monomeric U-box domain from E4B The AAA-ATPase VCP/p97 promotes 53BP1 recruitment by removing L3MBTL1 from DNA double-strand breaks Imbalances in p97 co-factor interactions in human proteinopathy UBXD7 binds multiple ubiquitin ligases and implicates p97 in HIF1alpha turnover The tissue-specific Rep8/UBXD6 tethers p97 to the endoplasmic reticulum membrane for degradation of misfolded proteins TRIM21: a cytosolic Fc receptor with broad antibody isotype specificity Insights into muscle degeneration from heritable inclusion body myopathies Myosin chaperones The ER-bound RING finger protein 5 (RNF5/RMA1) causes degenerative myopathy in transgenic mice and is deregulated in inclusion body myositis Cardiac-Restricted Expression of VCP/TER94 RNAi or Disease Alleles Perturbs Drosophila Heart Structure and Impairs Function.Regulation of protein quality control by UBE4B and LSD1 through p53-mediated transcription The UNC-45 chaperone is critical for establishing myosin-based myofibrillar organization and cardiac contractility in the Drosophila heart model The Myosin Chaperone UNC-45 Is Organized in Tandem Modules to Support Myofilament Formation in C. elegans The ubiquitin-selective chaperone Cdc48/p97 associates with Ubx3 to modulate monoubiquitylation of histone H2B.Cdc48/p97 mediates UV-dependent turnover of RNA Pol II.Mutations in the Human AAA+ Chaperone p97 and Related Diseases Structure-activity relationship study reveals ML240 and ML241 as potent and selective inhibitors of p97 ATPase Reversible inhibitor of p97, DBeQ, impairs both ubiquitin-dependent and autophagic protein clearance pathways The requirement for Cdc48/p97 in nuclear protein quality control degradation depends on the substrate and correlates with substrate insolubility The p97/VCP ATPase is critical in muscle atrophy and the accelerated degradation of muscle proteins Endolysosomal sorting of ubiquitylated caveolin-1 is regulated by VCP and UBXD1 and impaired by VCP disease mutations Heat-shock protein 90alpha1 is required for organized myofibril assembly in skeletal muscles of zebrafish embryos.VCP is essential for mitochondrial quality control by PINK1/Parkin and this function is impaired by VCP mutations.Lack of developmental redundancy between Unc45 proteins in zebrafish muscle development Novel transcriptional profile in wrist muscles from cerebral palsy patients.The Caenorhabditis elegans Elongator complex regulates neuronal alpha-tubulin acetylation.Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP disease Knockdown and overexpression of Unc-45b result in defective myofibril organization in skeletal muscles of zebrafish embryos.Ancient origin of animal U-box ubiquitin ligases.Inclusion body myopathy, Paget's disease of the bone and fronto-temporal dementia: a disorder of autophagy Specific inhibition of p97/VCP ATPase and kinetic analysis demonstrate interaction between D1 and D2 ATPase domains The EuroBioBank Network: 10 years of hands-on experience of collaborative, transnational biobanking for rare diseases 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.The VCP/p97 system at a glance: connecting cellular function to disease pathogenesis.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.The UNC-45 myosin chaperone: from worms to flies to vertebrates.Liver cytochrome P450 3A endoplasmic reticulum-associated degradation: a major role for the p97 AAA ATPase in cytochrome P450 3A extraction into the cytosol.Neuronal remodeling and apoptosis require VCP-dependent degradation of the apoptosis inhibitor DIAP1.Quantitative cell-based protein degradation assays to identify and classify drugs that target the ubiquitin-proteasome system.

P2860

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P2860

The ubiquitin-selective chaperone CDC-48/p97 links myosin assembly to human myopathy.

http://www.wikidata.org/entity/Q40157250

description

2007 nî lūn-bûn

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2007年の論文

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2007年論文

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2007年論文

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2007年論文

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2007年論文

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2007年論文

@zh-tw

2007年论文

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2007年论文

@zh

2007年论文

@zh-cn

name

The ubiquitin-selective chaperone CDC-48/p97 links myosin assembly to human myopathy.

@en

type

label

The ubiquitin-selective chaperone CDC-48/p97 links myosin assembly to human myopathy.

@en

prefLabel

The ubiquitin-selective chaperone CDC-48/p97 links myosin assembly to human myopathy.

@en

P1433

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P1433

Nature Cell Biology

P1476

The ubiquitin-selective chaperone CDC-48/p97 links myosin assembly to human myopathy.

@en

P2093

Giuseppe Cassata

http://www.w3.org/2001/XMLSchema#string

Julien Mouysset

http://www.w3.org/2001/XMLSchema#string

Philipp Christoph Janiesch

http://www.w3.org/2001/XMLSchema#string

Roja Barikbin

http://www.w3.org/2001/XMLSchema#string

Sabine Krause

http://www.w3.org/2001/XMLSchema#string

P2860

Identification of CHIP, a novel tetratricopeptide repeat-containing protein that interacts with heat shock proteins and negatively regulates chaperone functions.

Complementary signaling pathways regulate the unfolded protein response and are required for C. elegans development

Function of the p97-Ufd1-Npl4 complex in retrotranslocation from the ER to the cytosol: dual recognition of nonubiquitinated polypeptide segments and polyubiquitin chains

Direct binding of ubiquitin conjugates by the mammalian p97 adaptor complexes, p47 and Ufd1-Npl4.

Autosomal dominant myopathy: missense mutation (Glu-706 --> Lys) in the myosin heavy chain IIa gene

Mechanisms underlying ubiquitination

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

Protein dislocation from the ER requires polyubiquitination and the AAA-ATPase Cdc48.

A novel ubiquitination factor, E4, is involved in multiubiquitin chain assembly.

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

P2888

P304

379-390

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P31

scholarly article

P356

10.1038/NCB1554

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P433

4

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P478

9

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P50

Hanns Lochmüller

P577

2007-03-18T00:00:00Z

http://www.w3.org/2001/XMLSchema#dateTime

P5875

6437351

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P698

17369820

http://www.w3.org/2001/XMLSchema#string

P921

molecular chaperones