DATASHEET
Host:
Rabbit
Target Protein:
NPC2
Immunogen Range:
20-80/151
Clonality:
Polyclonal
Isotype:
IgG
Source:
KLH conjugated synthetic peptide derived from human Niemann Pick C2
Purification:
Purified by Protein A.
Storage Buffer:
0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Storage:
Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
Background:
NPC2 is a secreted protein mapping against gene 14q24.3 (1,2). NPC2 regulates the lipid composition of sperm membranes during maturation in the epididymis(1,2). Mutations in the NPC2 gene may cause Nieman-Pick type C2 disease and frontal lobe atrophy (1,2,3). Nieman-Pick type C2 is a fatal hereditary disease characterized by defective lysosome release of cholesterol (3). The disease is caused by HE1 deficiency, a lysosmal protein proven to be undetectable in fibroblasts from NPC2 patients (3). This differentiates NPC2 from NPC1, as NPC1 has HE1 protein present (3).
VALIDATION IMAGES
Mouse Lung lysates probed with NPC2 Polyclonal Antibody, Unconjugated (bs-11736R) at 1:300 dilution and 4˚C overnight incubation. Followed by conjugated secondary antibody incubation at 1:10000 for 60 min at 37˚C.
Lane 1: Recombinant human NPC2 protein, N-His(bs-42193P) probed with NPC2 Polyclonal Antibody, Unconjugated (bs-11736R) at 1:1000 dilution and 4°C overnight incubation. Followed by conjugated secondary antibody incubation at 1:20000 for 60 min at 37˚C.