Saposin C Protects Glucocerebrosidase against 伪-Synuclein Inhibition

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• 作者：Thai Leong Yap ; James M. Gruschus ; Arash Velayati ; Ellen Sidransky ; Jennifer C. Lee
• 刊名：Biochemistry
• 出版年：2013
• 出版时间：October 15, 2013
• 年：2013
• 卷：52
• 期：41
• 页码：7161-7163
• 全文大小：227K
• 年卷期：v.52,no.41(October 15, 2013)
• ISSN：1520-4995

文摘

Mutations in GBA1, the gene for glucocerebrosidase (GCase), are genetic risk factors for Parkinson disease (PD). 伪-Synuclein (伪-Syn), a protein implicated in PD, interacts with GCase and efficiently inhibits enzyme activity. GCase deficiency causes the lysosomal storage disorder Gaucher disease (GD). We show that saposin C (Sap C), a protein vital for GCase activity in vivo, protects GCase against 伪-syn inhibition. Using nuclear magnetic resonance spectroscopy, site-specific fluorescence, and F枚rster energy transfer probes, Sap C was observed to displace 伪-syn from GCase in solution and on lipid vesicles. Our results suggest that Sap C might play a crucial role in GD-related PD.