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

Abstract：

: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 protein 43 (TDP-43) was recently identified as a major disease protein in the ubiquitin-positive inclusions of sporadic and familial FTLD-U. To determine whether the ubiquitin pathology associated with mutations in VCP is characterized by the accumulation of TDP-43, we analyzed TDP-43 in the CNS pathology of five patients with VCP gene mutations. Accumulations of TDP-43 colocalized with ubiquitin pathology in inclusion body myopathy and Paget disease of bone, including both intranuclear inclusions and dystrophic neurites. Similar to FTLD-U, phosphorylated TDP-43 was detected only in insoluble brain extracts from affected brain regions. Identification of TDP-43, but not VCP, within ubiquitin-positive inclusions supports the hypothesis that VCP gene mutations lead to a dominant negative loss or alteration of VCP function culminating in impaired degradation of TDP-43. TDP-43 is a common pathologic substrate linking a variety of distinct patterns of FTLD-U pathology caused by different genetic alterations.

journal_name

J Neuropathol Exp Neurol

journal_title

Journal of neuropathology and experimental neurology

authors

Neumann M,Mackenzie IR,Cairns NJ,Boyer PJ,Markesbery WR,Smith CD,Taylor JP,Kretzschmar HA,Kimonis VE,Forman MS

doi

10.1097/nen.0b013e31803020b9

subject

Has Abstract

pub_date

2007-02-01 00:00:00

pages

152-7

issue

2

eissn

0022-3069

issn

1554-6578

pii

00005072-200702000-00007

journal_volume

66

pub_type

杂志文章

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