Article
Altered cofactor regulation with disease-associated p97/VCP mutations.
Proceedings of the National Academy of Sciences of the United States of America - 7 Apr 2015
Zhang Xiaoyi, Gui Lin, Zhang Xiaoyan, Bulfer Stacie L, Sanghez Valentina, Wong Daniel E, Lee YouJin, Lehmann Lynn, Lee James Siho, Shih Pei-Yin, Lin Henry J, Iacovino Michelina, Weihl Conrad C, Arkin Michelle R, Wang Yanzhuang, Chou Tsui-Fen
Abstract excerpt
Dominant mutations in p97/VCP (valosin-containing protein) cause a rare multisystem degenerative disease with varied phenotypes that include inclusion body myopathy, Paget's disease of bone, frontotemporal dementia, and amyotrophic lateral sclerosis. p97 disease mutants have altered N-domain conformations, elevated ATPase activity, and altered cofactor association. We have now discovered a previously unidentified...
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