Niemann-Pick C1 Monoclonal / Unconjugated / 8D10B6
Niemann-Pick C1 Monoclonal / Unconjugated / 8D10B6
Product Details
| Supplier | ||
|---|---|---|
| Catalog #: | NBP2-37735-0.025ml (View supplier product page) | |
| Size | 25 μl | |
| Price | $99.00 | |
| Antigen | Niemann-Pick C1 | |
| Clone | 8D10B6 | |
| Host | Mouse | |
| Isotype | IgG1 | |
| Conjugate | Unconjugated | |
| Target Species | Human | |
| Applications | WB, ELISA, IHC |
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] |
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Citations
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