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NPC1 rabbit pAb
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ES14463
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50 µL $148.00
100 µL $248.00
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Overview

Product name: NPC1 rabbit pAb
Reactivity: Human; Mouse
Source: Rabbit
Dilutions: WB 1：500-2000
Immunogen: Synthesized peptide derived from human NPC1 AA range: 1073-1123
Storage: -20°C/1 year
Clonality: Polyclonal
Isotype: IgG
Concentration: 1 mg/ml
Molecular Weight: 141kD
GeneID: 4864
Human Swiss-Prot No: O15118
Cellular localization: Late endosome membrane ; Multi-pass membrane protein . Lysosome membrane ; Multi-pass membrane protein .
Background: 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],