Ontology highlight
ABSTRACT:
SUBMITTER: Li X
PROVIDER: S-EPMC5018801 | biostudies-literature | 2016 Sep
REPOSITORIES: biostudies-literature
Li Xiaochun X Saha Piyali P Li Jian J Blobel Günter G Pfeffer Suzanne R SR
Proceedings of the National Academy of Sciences of the United States of America 20160822 36
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 ...[more]