| Product Name | Anti-NPC1 antibody-RBITC |
|---|---|
| Antibody Type | Primary Antibodies |
| Immunogen | Polypeptide |
| Conjugation | RBITC |
| Clonality | Polyclonal |
|---|---|
| Isotype | IgG |
| Host Species | Rabbit |
| Tested Applications | IFFC |
| IF:1:50-1:200 |
|
| Species Reactivity | HumanMouseRat |
| Concentration | 1mg/ml |
| Purification | Protein A |
| Gene Symbol | NPC1 |
|---|---|
| Gene Synonyms | NPC POGZ SLC65A1 |
| Gene Full Name | NPC intracellular cholesterol transporter 1 |
| Gene 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 compartments.[provided by RefSeq, Aug 2009] |
| Molecular Weight(MW) | 142kDa |
| Cellular Localization | Endosome, Lysosome, Membrane |
| SwissProt ID | O15118 |
|---|
| Application Notes | IF:1:50-1:200 |
|---|
| Form | Liquid |
|---|---|
| Storage Instructions | Shipped at 4°C. Store at +4°C short term (1-2 weeks). Store at -20°C long term. Avoid freeze / thaw cycle. |
| Storage Buffer | Purified antibody in PBS with 0.05% sodium azide. |
