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Article

TDP-43 in the ubiquitin pathology of frontotemporal dementia with VCP gene mutations.

Journal of neuropathology and experimental neurology - 1 Feb 2007

Neumann Manuela, Mackenzie Ian R, Cairns Nigel J, Boyer Philip J, Markesbery William R, Smith Charles D, Taylor J Paul, Kretzschmar Hans A, Kimonis Virginia E, Forman Mark S

Abstract excerpt

Frontotemporal dementia with inclusion body myopathy and Paget disease of bone is a rare, autosomal-dominant disorder caused by mutations in the gene valosin-containing protein (VCP). The CNS pathology is characterized by a novel pattern of ubiquitin pathology distinct from sporadic and familial frontotemporal lobar degeneration with ubiquitin-positive inclusions (FTLD-U) without VCP mutations. TAR DNA binding...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

Identifiers

PMID: 17279000
DOI: 10.1097/nen.0b013e31803020b9

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.

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TDP-43 in the ubiquitin pathology of frontotemporal dementia with VCP gene mutations.PMID 17279000Read ↗

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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 ↗

TDP-43-Positive White Matter Pathology in Frontotemporal Lobar Degeneration With Ubiquitin-Positive InclusionsSemantic proximity Read ↗

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