| Overview |
| bs-11736R-BF647 |
| NPC2/Niemann Pick C2 Polyclonal Antibody, AbBy Fluor® 647 Conjugated |
| WB |
| Mouse |
| Human, Rat, Cow, Sheep |
| Specifications |
| AbBy Fluor® 647 |
| Rabbit |
| KLH conjugated synthetic peptide derived from human Niemann Pick C2 |
| 20-80/151 |
| Polyclonal |
| IgG |
| 1ug/ul |
| Purified by Protein A. |
| Aqueous buffered solution containing 0.01M TBS (pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles. |
| Target |
| Cytoplasm, Secreted |
| EDDM1; Epididymal protein 1; Epididymal secretory protein; Epididymal secretory protein E1; HE1; Human epididymis-specic protein 1; Niemann-Pick disease type C2; Niemann-Pick disease type C2 protein; NPC2; NPC2_HUMAN; Tissue specic secretory protein. |
| 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). |
| Application Dilution |
| WB |
1:300-5000 |
Powered by Bioz