Article
Histone deacetylase inhibitors increase glucocerebrosidase activity in Gaucher disease by modulation of molecular chaperones.
Proceedings of the National Academy of Sciences of the United States of America - 15 Jan 2013
Yang Chunzhang, Rahimpour Shervin, Lu Jie, Pacak Karel, Ikejiri Barbara, Brady Roscoe O, Zhuang Zhengping
Abstract excerpt
Gaucher disease is caused by mutations of the GBA gene that encodes the lysosomal enzyme glucocerebrosidase (GCase). GBA mutations often result in protein misfolding and premature degradation, but usually exert less effect on catalytic activity. In this study, we identified the molecular mechanism by which histone deacetylase inhibitors increase the quantity and activity of GCase. Specifically, these inhibitors...
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