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http://purl.uniprot.org/citations/27551080http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/27551080http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/27551080http://www.w3.org/2000/01/rdf-schema#comment"Export of LDL-derived cholesterol from lysosomes requires the cooperation of the integral membrane protein Niemann-Pick C1 (NPC1) and a soluble protein, Niemann-Pick C2 (NPC2). Mutations in the genes encoding these proteins lead to Niemann-Pick disease type C (NPC). NPC2 binds to NPC1's second (middle), lumenally oriented domain (MLD) and transfers cholesterol to NPC1's N-terminal domain (NTD). Here, we report the 2.4-Å resolution crystal structure of a complex of human NPC1-MLD and NPC2 bearing bound cholesterol-3-O-sulfate. NPC1-MLD uses two protruding loops to bind NPC2, analogous to its interaction with the primed Ebola virus glycoprotein. Docking of the NPC1-NPC2 complex onto the full-length NPC1 structure reveals a direct cholesterol transfer tunnel between NPC2 and NTD cholesterol binding pockets, supporting the "hydrophobic hand-off" cholesterol transfer model."xsd:string
http://purl.uniprot.org/citations/27551080http://purl.org/dc/terms/identifier"doi:10.1073/pnas.1611956113"xsd:string
http://purl.uniprot.org/citations/27551080http://purl.org/dc/terms/identifier"doi:10.1073/pnas.1611956113"xsd:string
http://purl.uniprot.org/citations/27551080http://purl.uniprot.org/core/author"Li J."xsd:string
http://purl.uniprot.org/citations/27551080http://purl.uniprot.org/core/author"Li J."xsd:string
http://purl.uniprot.org/citations/27551080http://purl.uniprot.org/core/author"Li X."xsd:string
http://purl.uniprot.org/citations/27551080http://purl.uniprot.org/core/author"Li X."xsd:string
http://purl.uniprot.org/citations/27551080http://purl.uniprot.org/core/author"Pfeffer S.R."xsd:string
http://purl.uniprot.org/citations/27551080http://purl.uniprot.org/core/author"Pfeffer S.R."xsd:string
http://purl.uniprot.org/citations/27551080http://purl.uniprot.org/core/author"Blobel G."xsd:string
http://purl.uniprot.org/citations/27551080http://purl.uniprot.org/core/author"Blobel G."xsd:string
http://purl.uniprot.org/citations/27551080http://purl.uniprot.org/core/author"Saha P."xsd:string
http://purl.uniprot.org/citations/27551080http://purl.uniprot.org/core/author"Saha P."xsd:string
http://purl.uniprot.org/citations/27551080http://purl.uniprot.org/core/date"2016"xsd:gYear
http://purl.uniprot.org/citations/27551080http://purl.uniprot.org/core/date"2016"xsd:gYear
http://purl.uniprot.org/citations/27551080http://purl.uniprot.org/core/name"Proc. Natl. Acad. Sci. U.S.A."xsd:string
http://purl.uniprot.org/citations/27551080http://purl.uniprot.org/core/name"Proc. Natl. Acad. Sci. U.S.A."xsd:string
http://purl.uniprot.org/citations/27551080http://purl.uniprot.org/core/pages"10079-10084"xsd:string
http://purl.uniprot.org/citations/27551080http://purl.uniprot.org/core/pages"10079-10084"xsd:string
http://purl.uniprot.org/citations/27551080http://purl.uniprot.org/core/title"Clues to the mechanism of cholesterol transfer from the structure of NPC1 middle lumenal domain bound to NPC2."xsd:string
http://purl.uniprot.org/citations/27551080http://purl.uniprot.org/core/title"Clues to the mechanism of cholesterol transfer from the structure of NPC1 middle lumenal domain bound to NPC2."xsd:string
http://purl.uniprot.org/citations/27551080http://purl.uniprot.org/core/volume"113"xsd:string
http://purl.uniprot.org/citations/27551080http://purl.uniprot.org/core/volume"113"xsd:string