Article
Mutant glucocerebrosidase in Gaucher disease recruits Hsp27 to the Hsp90 chaperone complex for proteasomal degradation.
Proceedings of the National Academy of Sciences of the United States of America - 27 Jan 2015
Yang Chunzhang, Wang Herui, Zhu Dongwang, Hong Christopher S, Dmitriev Pauline, Zhang Chao, Li Yan, Ikejiri Barbara, Brady Roscoe O, Zhuang Zhengping
Abstract excerpt
Gaucher disease is caused by mutations of the GBA1 gene, which encodes the lysosomal anchored gluococerebrosidase (GCase). GBA1 mutations commonly result in protein misfolding, abnormal chaperone recognition, and premature degradation, but are less likely to affect catalytic activity. In the present study, we demonstrate that the Hsp90/HOP/Cdc37 complex recruits Hsp27 after recognition of GCase mutants with...
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