1nep

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[[Image:1nep.gif|left|200px]]
[[Image:1nep.gif|left|200px]]
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{{Structure
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|PDB= 1nep |SIZE=350|CAPTION= <scene name='initialview01'>1nep</scene>, resolution 1.70&Aring;
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The line below this paragraph, containing "STRUCTURE_1nep", creates the "Structure Box" on the page.
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|LIGAND= <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene>, <scene name='pdbligand=PO4:PHOSPHATE+ION'>PO4</scene>
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{{STRUCTURE_1nep| PDB=1nep | SCENE= }}
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|RESOURCES=<span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1nep FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1nep OCA], [http://www.ebi.ac.uk/pdbsum/1nep PDBsum], [http://www.rcsb.org/pdb/explore.do?structureId=1nep RCSB]</span>
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'''Crystal Structure Analysis of the Bovine NPC2 (Niemann-Pick C2) Protein'''
'''Crystal Structure Analysis of the Bovine NPC2 (Niemann-Pick C2) Protein'''
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[[Category: Lobel, P.]]
[[Category: Lobel, P.]]
[[Category: Stock, A M.]]
[[Category: Stock, A M.]]
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[[Category: bnpc2]]
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[[Category: Bnpc2]]
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[[Category: cholesterol]]
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[[Category: Cholesterol]]
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[[Category: ldl]]
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[[Category: Ldl]]
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[[Category: niemann-pick c2]]
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[[Category: Niemann-pick c2]]
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[[Category: npc2]]
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[[Category: Npc2]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sat May 3 02:26:36 2008''
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sun Mar 30 22:29:19 2008''
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Revision as of 23:26, 2 May 2008

Image:1nep.gif

Template:STRUCTURE 1nep

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. 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 Sat May 3 02:26:36 2008

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