1nep: Difference between revisions

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[[Image:1nep.gif|left|200px]]
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[[Image:1nep.png|left|200px]]


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{{STRUCTURE_1nep|  PDB=1nep  |  SCENE=  }}  
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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===




==Overview==
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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.
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{{ABSTRACT_PUBMED_12591954}}


==About this Structure==
==About this Structure==
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[[Category: Niemann-pick c2]]
[[Category: Niemann-pick c2]]
[[Category: Npc2]]
[[Category: Npc2]]
''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sat May  3 02:26:36 2008''
 
''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Tue Jul 29 06:32:24 2008''

Revision as of 03:32, 29 July 2008

File:1nep.png

Template:STRUCTURE 1nep

Crystal Structure Analysis of the Bovine NPC2 (Niemann-Pick C2) Protein

Template:ABSTRACT PUBMED 12591954

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

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