Niemann-Pick C1 / Unconjugated /
Niemann-Pick C1 / Unconjugated /
Product Details
| Supplier | Biorbyt | |
|---|---|---|
| Catalog #: | orb31550-400ul (View supplier product page) | |
| Size | 400 μl | |
| Price | $383.00 | |
| Antigen | Niemann-Pick C1 | |
| Clone | ||
| Host | Rabbit | |
| Isotype | IgG | |
| Conjugate | Unconjugated | |
| Target Species | Human | |
| Applications | ELISA, WB, IHC-P, IF, ICC | |
| 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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Images
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Immunofluorescense analysis of 293 cell using NPC1 antibody (primary antibody dilution at: 1:10-50), Immunohistochemical staining of paraffin embedded human kidney tissue using NPC1 antibody (primary antibody dilution at: 1:10-50), Western blot analysis of NCI-H460 cell line lysates (35ug/lane)using NPC1 antibody (primary antibody dilution at: 1:1000) |
Citations
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Reviews & Ratings
Purified Excitation and Emission Spectra
$383.00
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