1nep
From Proteopedia
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Crystal Structure Analysis of the Bovine NPC2 (Niemann-Pick C2) Protein
Overview
Niemann-Pick disease type C2 (NP-C2) is a fatal hereditary disease, characterized by accumulation of low-density lipoprotein-derived, cholesterol in lysosomes. Here we report the 1.7-A resolution crystal, structure of the cholesterol-binding protein deficient in this disease, NPC2, and the characterization of its ligand binding properties. Human, NPC2 binds the cholesterol analog dehydroergosterol with submicromolar, affinity at both acidic and neutral pH. NPC2 has an Ig-like fold, stabilized by three disulfide bonds. The structure of the bovine protein, reveals a loosely packed region penetrating from the surface into the, hydrophobic core that forms adjacent small cavities with a total volume of, approximately 160 A(3). We propose that this region represents the, incipient cholesterol-binding site that dilates to accommodate an, approximately 740-A(3) cholesterol molecule.
About this Structure
1NEP is a Single protein structure of sequence from Bos taurus with NAG and PO4 as ligands. Full crystallographic information is available from OCA.
Reference
Structure of a cholesterol-binding protein deficient in Niemann-Pick type C2 disease., Friedland N, Liou HL, Lobel P, Stock AM, Proc Natl Acad Sci U S A. 2003 Mar 4;100(5):2512-7. Epub 2003 Feb 18. PMID:12591954
Page seeded by OCA on Tue Nov 20 22:11:43 2007
Categories: Bos taurus | Single protein | Friedland, N. | Liou, H.L. | Lobel, P. | Stock, A.M. | NAG | PO4 | Bnpc2 | Cholesterol | Ldl | Niemann-pick c2 | Npc2
