Recombinant Human NPC2 /HE1 Protein (rh NPC2 / HE1) Glu 20 - Leu 151 (Accession # AAH02532) was produced in human 293 cells (HEK293) at ACROBiosystems.
rh NPC2 / HE1 is fused with a polyhistidine tag at the C-terminus, and has a calculated MW of 16.5 kDa. The predicted N-terminus is Glu 20. DTT-reduced Protein migrates as 20-24 kDa in SDS-PAGE due to glycosylation.
Less than 1.0 EU per μg of the rh NPC2 / HE1 by the LAL method.
>95% as determined by SDS-PAGE.
Lyophilized from 0.22 μm filtered solution in PBS, pH 7.4. Normally Mannitol or Trehalose are added as protectants before lyophilization.
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See Certificate of Analysis for reconstitution instructions and specific concentrations.
Avoid repeated freeze-thaw cycles.
No activity loss was observed after storage at:
In lyophilized state for 1 year (4oC); After reconstitution under sterile conditions for 3 months (-70oC).
Niemann-Pick disease type C2 protein (NPC2) is also known as epididymal secretory protein E1 and human epididymis-specific protein 1 (HE1). NPC2 is a 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. Also,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. Furthermore,the secreted form of NCP2 regulates biliary cholesterol secretion via stimulation of ABCG5/ABCG8-mediated cholesterol transport.
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