Article
3.3 Å structure of Niemann-Pick C1 protein reveals insights into the function of the C-terminal luminal domain in cholesterol transport.
Proceedings of the National Academy of Sciences of the United States of America - 22 Aug 2017
Li Xiaochun, Lu Feiran, Trinh Michael N, Schmiege Philip, Seemann Joachim, Wang Jiawei, Blobel Günter
Abstract excerpt
Niemann-Pick C1 (NPC1) and NPC2 proteins are indispensable for the export of LDL-derived cholesterol from late endosomes. Mutations in these proteins result in Niemann-Pick type C disease, a lysosomal storage disease. Despite recent reports of the NPC1 structure depicting its overall architecture, the function of its C-terminal luminal domain (CTD) remains poorly understood even though 45% of NPC disease-causing...
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