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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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| Main Authors: | , , , , |
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| Format: | Artigo |
| Sprog: | Inglês |
| Udgivet: |
2007
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| Fag: | |
| Online adgang: | 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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