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

Valosin-containing protein and the pathogenesis of frontotemporal dementia associated with inclusion body myopathy. Research Abstract Details 

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Valosin-containing protein and the pathogenesis of frontotemporal dementia associated with inclusion body myopathy. Abstract Text:

Jake B Guinto,Gillian P Ritson,J Paul Taylor,Mark S Forman,

Frontotemporal dementia with inclusion body myopathy and Paget's disease of bone (IBMPFD) 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 without VCP mutations. Yet, the ubiquitin-positive inclusions in IBMPFD also stain for TAR DNA binding protein, a feature that links this rare disease with the pathology associated with the majority of sporadic FTD as well as disease resulting from different genetic alterations. VCP, a member of the AAA-ATPase gene family, associates with a plethora of protein adaptors to perform a variety of cellular processes including Golgi assembly/disassembly and regulation of the ubiquitin-proteasome system. However, the mechanism whereby mutations in VCP lead to CNS, muscle, and bone disease is largely unknown. In this report, we review current literature on IBMPFD, focusing on the pathology of the disease and the biology of VCP with respect to IBMPFD.

Valosin-containing protein and the pathogenesis of frontotemporal dementia associated with inclusion body myopathy. Publishing Authors By Initials

JB Guinto,GP Ritson,JP Taylor,MS Forman,

For similar musculoskeletal diseases: bone diseases: osteitis deformans research abstracts see: musculoskeletal diseases: bone diseases: osteitis deformans research

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Valosin-containing protein and the pathogenesis of frontotemporal dementia associated with inclusion body myopathy. Journal Published:

PUBLICATION TYPE: Review

Journal: Acta neuropathologica

VOLUME: 114

Page Numbers: 55-61

Journal Abbreviation: Acta Neuropathol.

ISSN: 0001-6322

DAY: 25

MONTH: 04

YEAR: 2007

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

Number of References: 63

LANGUAGE: eng

NlmUniqueID: 412041

Valosin-containing protein and the pathogenesis of frontotemporal dementia associated with inclusion body myopathy. Keywords Mesh Terms:

KEYWORDS: Osteitis Deformans

MESH TERMS: pathology

Chemical & Substance for Abstract: Valosin-containing protein and the pathogenesis of frontotemporal dementia associated with inclusion body myopathy. Information

Substance Name: Adenosine Triphosphatases

Registry Number: EC 3.6.1.-

Grant and Affiliation Information for Valosin-containing protein and the pathogenesis of frontotemporal dementia associated with inclusion body myopathy.

AFFILIATION: Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, 422 Curie Blvd., 605B Stellar-Chance Building, Philadelphia, PA, 19104-6140, USA.

Country: Germany

AGENCY: United States NINDS

GRANT: NS053825

ACRONYM: NS

MEDLINETA: Acta Neuropathol

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