StoragePBS with 0.02% sodium azide and 50% glycerol pH 7.3 , -20℃ for 24 months (Avoid repeated freeze / thaw cycles.)
Species ReactivityHuman, Mouse ,Rat
PurificationImmunogen affinity purified
ImmunogenNiemann-Pick disease, type C2
Recommended dilutionWB : 1:200-1:2000
Product Description specificalIntracellular 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.