期刊
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS
卷 1685, 期 1-3, 页码 22-27出版社
ELSEVIER
DOI: 10.1016/j.bbalip.2004.08.008
关键词
trafficking; Niemann-Pick C protein; lipid
资金
- NIDDK NIH HHS [DK49564, DK54320] Funding Source: Medline
Niemann-Pick C 1 (NPC1) is a large integral membrane glycoprotein that resides in late endosomes, whereas NPC2 is a small soluble protein found in the lumen of lysosomes. Mutations in either NPC1 or NPC2 result in aberrant lipid transport from endocytic compartments, which results in lysosomal storage of a complex mixture of lipids, primarily cholesterol and glycosphingolipids. What are the biological functions of the NPC1 and NPC2 proteins? Here we review what is known about the intracellular itinerary of these two proteins as they facilitate lipid transport. We propose that the intracellular trafficking patterns of these proteins will provide clues about their function. (C) 2004 Elsevier B.V. All rights reserved.
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