Article
Structural and Functional Analysis of Disease-Linked p97 ATPase Mutant Complexes.
International journal of molecular sciences - 28 Jul 2021
Nandi Purbasha, Li Shan, Columbres Rod Carlo A, Wang Feng, Williams Dewight R, Poh Yu-Ping, Chou Tsui-Fen, Chiu Po-Lin
Abstract excerpt
IBMPFD/ALS is a genetic disorder caused by a single amino acid mutation on the p97 ATPase, promoting ATPase activity and cofactor dysregulation. The disease mechanism underlying p97 ATPase malfunction remains unclear. To understand how the mutation alters the ATPase regulation, we assembled a full-length p97R155H with its p47 cofactor and first visualized their structures using single-particle cryo-EM. More than...
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