Article
VCP/p97 is essential for maturation of ubiquitin-containing autophagosomes and this function is impaired by mutations that cause IBMPFD.
Autophagy - 1 Feb 2010
Tresse Emilie, Salomons Florian A, Vesa Jouni, Bott Laura C, Kimonis Virginia, Yao Tso-Pang, Dantuma Nico P, Taylor J Paul
Abstract excerpt
VCP (VCP/p97) is a ubiquitously expressed member of the AAA(+)-ATPase family of chaperone-like proteins that regulates numerous cellular processes including chromatin decondensation, homotypic membrane fusion and ubiquitin-dependent protein degradation by the proteasome. Mutations in VCP cause a multisystem degenerative disease consisting of inclusion body myopathy, Paget disease of bone, and frontotemporal...
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