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• W2744732181 abstract "Significance The Niemann–Pick C1 (NPC1) protein is responsible for transporting LDL-derived cholesterol out of late endosomes. Mutations in NPC1 lead to the fatal Niemann–Pick Type C disease. We present here an improved structure of an NPC1 protein at 3.3 Å and decipher details of its C-terminal luminal domain (CTD), which could not be resolved in previous structures. In particular, a loop stabilized by a pair of disulfide bonds in the CTD binds to the N-terminal domain through a loop–loop interaction. We show that this interaction is important for cholesterol transport in cultured cells. Together, our data provide insights related to the molecular mechanism of NPC1 activity and Niemann–Pick Type C disease." @default.
• W2744732181 created "2017-08-17" @default.
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• W2744732181 date "2017-08-07" @default.
• W2744732181 modified "2023-10-10" @default.
• W2744732181 title "3.3 Å structure of Niemann–Pick C1 protein reveals insights into the function of the C-terminal luminal domain in cholesterol transport" @default.
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• W2744732181 doi "https://doi.org/10.1073/pnas.1711716114" @default.
• W2744732181 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/5576846" @default.
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