期刊
FEBS LETTERS
卷 584, 期 9, 页码 1700-1712出版社
WILEY
DOI: 10.1016/j.febslet.2009.10.021
关键词
Endosome; GM2-activator; Lysosome; Niemann-Pick disease, type C2-protein; Saposin; Sphingolipid activator protein
资金
- DFG [SFB 645, TRR 83]
- European Community [202272]
The constitutive degradation of membrane components takes place in the acidic compartments of a cell, the endosomes and lysosomes. Sites of lipid degradation are intralysosomal membranes that are formed in endosomes, where the lipid composition is adjusted for degradation. Cholesterol is sorted out of the inner membranes, their content in bis(monoacylglycero)phosphate increases, and, most likely, sphingomyelin is degraded to ceramide. Together with endosomal and lysosomal lipid-binding proteins, the Niemann-Pick disease, type C2-protein, the GM2-activator, and the saposins sap-A, -B, -C, and -D, a suitable membrane lipid composition is required for degradation of complex lipids by hydrolytic enzymes. (C) 2009 Federation of European Biochemical Societies. Published by Elsevier B. V. All rights reserved.
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