Niemann-Pick C1 / Unconjugated / 8D10G3
Niemann-Pick C1 / Unconjugated / 8D10G3
Product Details
Supplier | ||
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Catalog #: | NBP2-37369 (View supplier product page) | |
Size | 100 μl | |
Price | ||
Antigen | Niemann-Pick C1 | |
Clone | 8D10G3 | |
Host | Mouse | |
Isotype | IgG1 | |
Conjugate | Unconjugated | |
Target Species | Human | |
Applications | ELISA, FC, WB, CyTOF |
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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