Npc2
Domain
Binds cholesterol in a hydrophobic pocket; there are no hydrogen bonds between the sterol and the protein.
Function
Intracellular cholesterol transporter which acts in concert with NPC1 and plays an important role in the egress of cholesterol from the lysosomal compartment (PubMed:12591949, PubMed:17018531, PubMed:21315718, PubMed:26296895). 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 (By similarity). The secreted form of NCP2 regulates biliary cholesterol secretion via stimulation of ABCG5/ABCG8-mediated cholesterol transport (PubMed:21315718).
Post-translational modifications
N-glycosylated.
Sequence Similarities
Belongs to the NPC2 family.
Tissue Specificity
Detected in liver and bile (PubMed:21315718). Detected in epididymis (at protein level). Detected in caput epididymis, corpus epididymis, cauda epididymis and ovary (PubMed:10863096).
Cellular localization
- Secreted
- Endoplasmic reticulum
- Lysosome
- Interaction with cell-surface M6PR mediates endocytosis and targeting to lysosomes.
Alternative names
NPC intracellular cholesterol transporter 2, Epididymal secretory protein E1, Niemann Pick type C2 protein homolog, mE1, Npc2