Journal
CLINICA CHIMICA ACTA
Volume 429, Issue -, Pages 69-75Publisher
ELSEVIER SCIENCE BV
DOI: 10.1016/j.cca.2013.11.026
Keywords
NPC1; NPC2; Cholesterol; ORP5; Atherosclerosis
Categories
Funding
- National Natural Sciences Foundation of China [81070220, 81170278]
- Aid Program for Science and Technology Innovative Research Team in Higher Educational Institutions of Human Province, China [2008-244]
- construct program of the key discipline in Hunan Province
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Post-lysosomal cholesterol trafficking is an important, but poorly understood process that is essential to maintain lipid homeostasis. Niemann-Pick type Cl (NPC1), an integral membrane protein on the limiting membrane of late endosome/lysosome (LE/LY), is known to accept cholesterol from NPC2 and then mediate cholesterol transport from LE/LY to endoplasmic reticulum (ER) and plasma membrane in a vesicle- or oxysterol-binding protein (OSBP)-related protein 5 (ORP5)-dependent manner. Mutations in the NPC1 gene can be found in the majority of NPC patients, who accumulate massive amounts of cholesterol and other lipids in the LE/LY due to a defect in intracellular lipid trafficking. Liver X receptor (LXR) is the major positive regulator of NPC1 expression. Atherosclerosis is the pathological basis of coronary heart disease, one of the major causes of death worldwide. NPC1 has been shown to play a critical role in the atherosclerotic progression. In this review, we have summarized the role of NPC1 in regulating intracellular cholesterol trafficking and atherosclerosis. (C) 2013 Elsevier B.V. All rights reserved.
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