Celastrol increases glucocerebrosidase activity in Gaucher disease by modulating molecular chaperones

Gaucher disease is caused by mutations in the glucosidase, beta, acid gene that encodes glucocerebrosidase (GCase). Glucosidase, beta, acid mutations often cause protein misfolding and quantitative loss of GCase. In the present study, we found that celastrol, an herb derivative with known anticancer...

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Veröffentlicht in:	Proceedings of the National Academy of Sciences - PNAS 2014-01, Vol.111 (1), p.249-254
Hauptverfasser:	Yang, Chunzhang, Swallows, Cody L., Zhang, Chao, Lu, Jie, Xiao, Hongbin, Brady, Roscoe O., Zhuang, Zhengping
Format:	Artikel
Sprache:	eng
Schlagworte:	Adaptor Proteins, Signal Transducing - metabolism Apoptosis Regulatory Proteins Biological Sciences Catalysis Catalytic activity Cell Cycle Proteins - metabolism Chaperonins - metabolism Enzymes Fibroblasts Fibroblasts - metabolism Gaucher disease Gaucher Disease - genetics Gaucher Disease - metabolism Gene Expression Regulation, Enzymologic - drug effects Genes Genetic disorders Genetics Glucosylceramidase - genetics Glucosylceramidase - metabolism Heat shock proteins Heat shock response HeLa Cells HSP70 Heat-Shock Proteins - metabolism HSP90 Heat-Shock Proteins - metabolism Humans Medical treatment Molecular chaperones Molecular Chaperones - chemistry Mutation Plant Preparations - pharmacology Protein Binding Protein Denaturation Protein Folding Real-Time Polymerase Chain Reaction RNA Interference Shock heating Triterpenes - pharmacology
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