NPC intracellular cholesterol transporter 2

Definition:

Intracellular cholesterol transporter which acts in concert with NPC1 and plays an important role in the egress of cholesterol from the lysosomal compartment. Unesterified cholesterol that has been released from LDLs in the lumen of the late endosomes/lysosomes is transferred by NPC2 to the cholesterol-binding pocket in the N-terminal domain of NPC1. May bind and mobilize cholesterol that is associated with membranes. NPC2 binds cholesterol with a 1:1 stoichiometry. Can bind a variety of sterols, including lathosterol, desmosterol and the plant sterols stigmasterol and beta-sitosterol. The secreted form of NCP2 regulates biliary cholesterol secretion via stimulation of ABCG5/ABCG8-mediated cholesterol transport (By similarity).

References:

[1]. Zhi Xu, et al. Regulation of sterol transport between membranes and NPC2. Biochemistry. 2008 Oct 21;47(42):11134-43. [Content Brief]

[2]. S Naureckiene, et al. Identification of HE1 as the second gene of Niemann-Pick C disease. Science. 2000 Dec 22;290(5500):2298-301. [Content Brief]

[3]. Xin Gong, et al. Structural Insights into the Niemann-Pick C1 (NPC1)-Mediated Cholesterol Transfer and Ebola Infection. Cell. 2016 Jun 2;165(6):1467-1478. [Content Brief]

[4]. Zane Berzina, et al. Niemann-Pick C2 protein regulates sterol transport between plasma membrane and late endosomes in human fibroblasts. Chem Phys Lipids. 2018 Jul;213:48-61. [Content Brief]

[5]. Heng-Ling Liou, et al. NPC2, the protein deficient in Niemann-Pick C2 disease, consists of multiple glycoforms that bind a variety of sterols. J Biol Chem. 2006 Dec 1;281(48):36710-23. [Content Brief]

[6]. Karim Chikh, et al. Niemann-Pick type C disease: subcellular location and functional characterization of NPC2 proteins with naturally occurring missense mutations. Hum Mutat. 2005 Jul;26(1):20-8. [Content Brief]

[7]. Rodney E Infante, et al. NPC2 facilitates bidirectional transfer of cholesterol between NPC1 and lipid bilayers, a step in cholesterol egress from lysosomes. Proc Natl Acad Sci U S A. 2008 Oct 7;105(40):15287-92. [Content Brief]

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