Cofactor regulation of p97/VCP disease mutants [Biochemistry]
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...
Source: Proceedings of the National Academy of Sciences - Category: Science Authors: Zhang, X., Gui, L., , Bulfer, S. L., Sanghez, V., Wong, D. E., Lee, Y., Lehmann, L., Lee, J. S., Shih, P.-Y., Lin, H. J., Iacovino, M., Weihl, C. C., Arkin, M. R., Wang, Y., Chou, T.-F. Tags: PNAS Plus Source Type: research