1nep

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Template:STRUCTURE 1nep

Contents

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

Template:ABSTRACT PUBMED 12591954

Function

[NPC2_BOVIN] Intracellular cholesterol transporter which acts in concert with NPC1 and plays an important role in the egress of cholesterol from the endosomal/lysosomal compartment. Both NPC1 and NPC2 function as the cellular 'tag team duo' (TTD) to catalyze the mobilization of cholesterol within the multivesicular environment of the late endosome (LE) to effect egress through the limiting bilayer of the LE. NPC2 binds unesterified cholesterol that has been released from LDLs in the lumen of the late endosomes/lysosomes and transfers it to the cholesterol-binding pocket of the N-terminal domain of NPC1. Cholesterol binds to NPC1 with the hydroxyl group buried in the binding pocket and is exported from the limiting membrane of late endosomes/ lysosomes to the ER and plasma membrane by an unknown mechanism. The secreted form of NCP2 regulates biliary cholesterol secretion via stimulation of ABCG5/ABCG8-mediated cholesterol transport.[1]

About this Structure

1nep is a 1 chain structure with sequence from Bos taurus. Full crystallographic information is available from OCA.

Reference

  • Friedland N, Liou HL, Lobel P, Stock AM. Structure of a cholesterol-binding protein deficient in Niemann-Pick type C2 disease. Proc Natl Acad Sci U S A. 2003 Mar 4;100(5):2512-7. Epub 2003 Feb 18. PMID:12591954 doi:10.1073/pnas.0437840100
  1. ↑ Xu Z, Farver W, Kodukula S, Storch J. Regulation of sterol transport between membranes and NPC2. Biochemistry. 2008 Oct 21;47(42):11134-43. doi: 10.1021/bi801328u. Epub 2008 Sep , 30. PMID:18823126 doi:http://dx.doi.org/10.1021/bi801328u

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