Ontology highlight
ABSTRACT:
SUBMITTER: Yang C
PROVIDER: S-EPMC4313839 | biostudies-literature | 2015 Jan
REPOSITORIES: biostudies-literature
Yang Chunzhang C Wang Herui H Zhu Dongwang D Hong Christopher S CS Dmitriev Pauline P Zhang Chao C Li Yan Y Ikejiri Barbara B Brady Roscoe O RO Zhuang Zhengping Z
Proceedings of the National Academy of Sciences of the United States of America 20150112 4
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 subsequent targeting of GCase mutant peptides to degradation mechanisms such as VCP and t ...[more]