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Article

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

Human molecular genetics - 15 Apr 2010

Ju Jeong-Sun, Weihl Conrad C

Abstract excerpt

Inclusion body myopathy associated with Paget's disease of the bone and fronto-temporal dementia (IBMPFD) is a progressive autosomal dominant disorder caused by mutations in p97/VCP (valosin-containing protein). p97/VCP is a member of the AAA+ (ATPase associated with a variety of activities) protein family and participates in multiple cellular processes. One particularly important role for p97/VCP is facilitating...

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Source

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Identifiers

PMID: 20410287
DOI: 10.1093/hmg/ddq157
PMCID: PMC2875057

Genes

VCP

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

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Inclusion body myopathy, Paget's disease of the bone and fronto-temporal dementia: a disorder of autophagy.PMID 20410287Read ↗

Impaired protein aggregate handling and clearance underlie the pathogenesis of p97/VCP-associated disease.Semantic proximity Read ↗

Recent advances in p97/VCP/Cdc48 cellular functions.Semantic proximity Read ↗

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

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

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

VCP disease associated with myopathy, Paget disease of bone and frontotemporal dementia: review of a unique disorder.Semantic proximity Read ↗

Transgenic mice expressing mutant forms VCP/p97 recapitulate the full spectrum of IBMPFD including degeneration in muscle, brain and boneSemantic proximity Read ↗

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

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

Autosomal Dominant Inclusion Body Myopathy, Paget Disease of Bone, and Frontotemporal DementiaSemantic proximity Read ↗

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