Niemann-Pick C1 / Unconjugated /
Niemann-Pick C1 / Unconjugated /
Product Details
| Supplier | Biorbyt | |
|---|---|---|
| Catalog #: | orb254120-100ul (View supplier product page) | |
| Size | 100 μl | |
| Price | $446.00 | |
| Antigen | Niemann-Pick C1 | |
| Clone | ||
| Host | Rabbit | |
| Isotype | IgG | |
| Conjugate | Unconjugated | |
| Target Species | Human, Mouse, Rat | |
| Applications | IHC-P | |
| Description | Rabbit polyclonal antibody to NPC1 |
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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Reviews & Ratings
Purified Excitation and Emission Spectra
$446.00
Supplier Page
Supplier Page
