Article
Valosin-containing protein and the pathogenesis of frontotemporal dementia associated with inclusion body myopathy.
Acta neuropathologica - 1 Jul 2007
Guinto Jake B, Ritson Gillian P, Taylor J Paul, Forman Mark S
Abstract excerpt
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...
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