Edit |   |
---|---|
Antigenic Specificity | NPC1 |
Clone | [8D10B6] |
Host Species | Mouse |
Reactive Species | human |
Isotype | IgG1 |
Format | unconjugated |
Size | 0.1 mL |
Concentration | n/a |
Applications | Western Blot (WB), Immunohistochemistry (IHC) |
Reviews / Ratings | If you have used this antibody, please help fellow researchers by submitting reviews to pAbmAbs and antYbuddY. |
Description | Entrez Summary: This gene encodes a large protein that resides in the limiting membrane of endosomes and lysosomes and mediates intracellular cholesterol trafficking via binding of cholesterol to its N-terminal domain. It is predicted to have a cytoplasmic C-terminus, 13 transmembrane domains, and 3 large loops in the lumen of the endosome - the last loop being at the N-terminus. This protein transports low-density lipoproteins to late endosomal/lysosomal compartments where they are hydrolized and released as free cholesterol. Defects in this gene cause Niemann-Pick type C disease, a rare autosomal recessive neurodegenerative disorder characterized by over accumulation of cholesterol and glycosphingolipids in late endosomal/lysosomal compar |
Immunogen | Immunogen: Purified recombinant fragment of human NPC1 (AA: 34-174) expressed in E Coli. |
Other Names | [NPC], [NPC] |
Gene, Accession # | [NPC1], Gene ID: 4864, NCBI: NP_000262.2, UniProt: O15118 |
Catalog # | MBS476448 |
Price | $335 |
Order / More Info | NPC1 Antibody from MYBIOSOURCE INC. |
Product Specific References | n/a |