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STRUCTURAL BASIS OF STEROL BINDING BY NPC2, A LYSOSOMAL PROTEIN DEFICIENT IN NIEMANN-PICK TYPE C2 DISEASE

NPC2 is a small lysosomal glycoprotein that binds cholesterol with submicromolar affinity. Deficiency in NPC2 is the cause of Niemann Pick type C2 disease, a fatal neurovisceral disorder characterized by accumulation of cholesterol in lysosomes. Here we report the crystal structure of bovine NPC2 bo...

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Détails bibliographiques
Auteurs principaux: Xu, Sujuan, Benoff, Brian, Liou, Heng-Ling, Lobel, Peter, Stock, Ann M.
Format: Artigo
Langue:Inglês
Publié: 2007
Sujets:
Accès en ligne:https://ncbi.nlm.nih.gov/pmc/articles/PMC3645284/
https://ncbi.nlm.nih.gov/pubmed/17573352
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M703848200
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