Species: | Rabbit |
Applications: | WB IHC IF |
Immunogen Range: | KLH conjugated synthetic peptide derived from human Niemann Pick C2 |
Clonality: | Polyclonal Antibody |
Isotype: | IgG |
GENE ID: | |
Swiss Prot: | |
Synonyms: | 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. |
Purification: | Purified by Protein A. |
Storage: | Aqueous buffered solution containing 100ug/ml BSA, 50% glycerol and 0.09% sodium azide. Store at -20℃ for 12 months |
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). |
Caculated MW: | / |
Observed MW: | Refer to Figures |
Applications: |
WB 1:100-1:1000 IHC 1:100-1:500 IF 1:50-1:200 |
Reacitivity: | Human, Mouse, Rat |