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Article

Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP disease.

The Journal of cell biology - 14 Dec 2009

Ju Jeong-Sun, Fuentealba Rodrigo A, Miller Sara E, Jackson Erin, Piwnica-Worms David, Baloh Robert H, Weihl Conrad C

Abstract excerpt

Mutations in valosin-containing protein (VCP) cause inclusion body myopathy (IBM), Paget's disease of the bone, and frontotemporal dementia (IBMPFD). Patient muscle has degenerating fibers, rimmed vacuoles (RVs), and sarcoplasmic inclusions containing ubiquitin and TDP-43 (TARDNA-binding protein...

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Source

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Identifiers

PMID: 20008565
DOI: 10.1083/jcb.200908115
PMCID: PMC2806317

Genes

VCP

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 2 Sept 2026. Prepared record publication-20008565-2026-09-02.2. Manifest publication-graph-cohort-2026-09-02.2.

Source receipt: PubMed via Helena Literature Corpus, retrieved 2 Sept 2026.

Related research

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Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP disease.PMID 20008565Read ↗

mTOR dysfunction contributes to vacuolar pathology and weakness in valosin-containing protein associated inclusion body myopathySemantic proximity Read ↗

Valosin Containing Protein Associated Fronto-Temporal Lobar Degeneration:Clinical Presentation, Pathologic Features and PathogenesisSemantic proximity Read ↗

Valosin containing protein associated inclusion body myopathy: abnormal vacuolization, autophagy and cell fusion in myoblasts.Semantic proximity Read ↗

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

Valosin-containing protein disease: inclusion body myopathy with Paget's disease of the bone and fronto-temporal dementia.Semantic proximity Read ↗

The multiple faces of valosin-containing protein-associated diseases: inclusion body myopathy with Paget's disease of bone, frontotemporal dementia, and amyotrophic lateral sclerosis.Semantic proximity Read ↗

Inclusion body myopathy, Paget's disease of the bone and fronto-temporal dementia: a disorder of autophagy.Semantic proximity Read ↗

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Valosin-containing protein and the pathogenesis of frontotemporal dementia associated with inclusion body myopathy.Semantic proximity Read ↗

IBMPFD Disease-Causing Mutant VCP/p97 Proteins Are Targets of Autophagic-Lysosomal Degradation.Semantic proximity Read ↗

CRISPR/Cas9-Mediated Constitutive Loss of VCP (Valosin-Containing Protein) Impairs Proteostasis and Leads to Defective Striated Muscle Structure and Function In Vivo.Semantic proximity Read ↗

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