Niemann-Pick C1 / Unconjugated / 1318A
Niemann-Pick C1 / Unconjugated / 1318A
Product Details
| Supplier | R&D Systems | |
|---|---|---|
| Catalog #: | MAB10105-100 (View supplier product page) | |
| Size | 100 μg | |
| Price | $409.00 | |
| Antigen | Niemann-Pick C1 | |
| Clone | 1318A | |
| Host | Rabbit | |
| Isotype | IgG1 | |
| Conjugate | Unconjugated | |
| Target Species | Human, Mouse, Rat | |
| Applications | WB, IHC, Knockout Validated | |
| Description | Human/Mouse/Rat Niemann-Pick Type C1/NPC1 Antibody |
About Niemann-Pick C1 and Purified
| Niemann-Pick C1 | 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] |
|---|
Citations
| Additional Sources |
|
|---|
Reviews & Ratings
Purified Excitation and Emission Spectra
$409.00
Supplier Page
Supplier Page
