ELISA/assay

NPC2 ELISA Kit (Human) (OKCD01269)

OKCD01269

96 Wells

Application Kit

ELISA Kits

NPC2 ELISA Kit (Human) (OKCD01269)

96 Wells

NPC2

EDDM1, Epididymal secretory protein E1, He1, HE1, Human epididymis-specific protein 1, MGC1333, Niemann-Pick disease type C2 protein, NP-C2

NM_006432.3

NP_006423

P61916

Human

Store as indicated in product manual.

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.3 Publications p>Manually curated information for which there is published experimental evidence. /p> a href="/manual/evidences#ECO:269">More… /a> /p> Manual assertion based on experiment iniRef.5"NPC2, the protein deficient in Niemann-Pick C2 disease, consists of multiple glycoforms that bind a variety of sterols." Liou H.L., Dixit S.S., Xu S., Tint G.S., Stock A.M., Lobel P. J. Biol. Chem. 281:3671-36723(26) [PubMed] [Europe PMC] [Abstract]Cited for: PROTEIN SEQUENCE OF N-TERMINUS, GLYCOSYLATION AT ASN-58 AND ASN-135, FUNCTION.Ref.9"Regulation of sterol transport between membranes and NPC2." Xu Z., Farver W., Kodukula S., Storch J. Biochemistry 47:11134-11143(28) [PubMed] [Europe PMC] [Abstract]Cited for: FUNCTION.Ref.1"NPC2 facilitates bidirectional transfer of cholesterol between NPC1 and lipid bilayers, a step in cholesterol egress from lysosomes." Infante R.E., Wang M.L., Radhakrishnan A., Kwon H.J., Brown M.S., Goldstein J.L. Proc. Natl. Acad. Sci. U.S.A. 15:15287-15292(28) [PubMed] [Europe PMC] [Abstract]Cited for: FUNCTION.

ELISA

Assay Methodology: Quantitative Sandwich Immunoassay

< 0.29 ng/mL

This assay has high sensitivity and excellent specificity for detection of Niemann Pick Disease Type C2 (NPC2). No significant cross-reactivity or interference between Niemann Pick Disease Type C2 (NPC2) and analogues was observed.

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