| Applications: | WB, IF, IHC-P |
| Species Reactivity: | Human |
| Isotype: | Rabbit Ig |
| Purification: | Affinity Purified Rabbit Polyclonal Antibody |
| Description: | This gene encodes a large protein that resides in thelimiting membrane of endosomes and lysosomes and mediatesintracellular cholesterol trafficking via binding of cholesterol toits N-terminal domain. It is predicted to have a cytoplasmicC-terminus, 13 transmembrane domains, and 3 large loops in thelumen of the endosome - the last loop being at the N-terminus. Thisprotein transports low-density lipoproteins to lateendosomal/lysosomal compartments where they are hydrolized andreleased as free cholesterol. Defects in this gene causeNiemann-Pick type C disease, a rare autosomal recessiveneurodegenerative disorder characterized by over accumulation ofcholesterol and glycosphingolipids in late endosomal/lysosomalcompartments. |
| Further Information: | This NPC1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 591-620 amino acids from the Central region of human NPC1. |
| Synonym: | Niemann-Pick C1 protein, NPC1 |
| Ascension Number: | O15118 |
| NCBI EntrezGene: | 4864 |
| Shipping Conditions: | Ship on cold packs |
| Storage: | Maintain refrigerated at 2-8 C for up to 2 weeks. For long term storage store at -20 C in small aliquots to prevent freeze-thaw cycles. |
| Usage: | Research use only |
