Late endosomal membranes rich in lysobisphosphatidic acid regulate cholesterol transport.
摘要：
The fate of free cholesterol released after endocytosis of low-density lipoproteins remains obscure. Here we report that late endosomes have a pivotal role in intracellular cholesterol transport. We find that in the genetic disease Niemann-Pick type C (NPC), and in drug-treated cells that mimic NPC, cholesterol accumulates in late endosomes and sorting of the lysosomal enzyme receptor is impaired. Our results show that the characteristic network of lysobisphosphatidic acid-rich membranes contained within multivesicular late endosomes regulates cholesterol transport, presumably by acting as a collection and distribution device. The results also suggest that similar endosomal defects accompany the anti-phospholipid syndrome and NPC.
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关键词：
Animals Humans Endosomes Lysosomes Fibroblasts Cells, Cultured Cell Line Intracellular Membranes Niemann-Pick Diseases Zinc
DOI：
10.1038/10084
被引量：
年份：
1999
Springer
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