Ontology highlight
ABSTRACT:
SUBMITTER: Kwon HJ
PROVIDER: S-EPMC2739658 | biostudies-literature | 2009 Jun
REPOSITORIES: biostudies-literature
Kwon Hyock Joo HJ Abi-Mosleh Lina L Wang Michael L ML Deisenhofer Johann J Goldstein Joseph L JL Brown Michael S MS Infante Rodney E RE
Cell 20090601 7
LDL delivers cholesterol to lysosomes by receptor-mediated endocytosis. Exit of cholesterol from lysosomes requires two proteins, membrane-bound Niemann-Pick C1 (NPC1) and soluble NPC2. NPC2 binds cholesterol with its isooctyl side chain buried and its 3beta-hydroxyl exposed. Here, we describe high-resolution structures of the N-terminal domain (NTD) of NPC1 and complexes with cholesterol and 25-hydroxycholesterol. NPC1(NTD) binds cholesterol in an orientation opposite to NPC2: 3beta-hydroxyl bu ...[more]