Article
Comprehensive structural characterization of the human AAA+ disaggregase CLPB in the apo- and substrate-bound states reveals a unique mode of action driven by oligomerization.
PLoS biology - 1 Feb 2023
Wu Damu, Liu Yan, Dai Yuhao, Wang Guopeng, Lu Guoliang, Chen Yan, Li Ningning, Lin Jinzhong, Gao Ning
Abstract excerpt
The human AAA+ ATPase CLPB (SKD3) is a protein disaggregase in the mitochondrial intermembrane space (IMS) and functions to promote the solubilization of various mitochondrial proteins. Loss-of-function CLPB mutations are associated with a few human diseases with neutropenia and neurological disorders. Unlike canonical AAA+ proteins, CLPB contains a unique ankyrin repeat domain (ANK) at its N-terminus. How CLPB...
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