NPC1 rabbit pAb - ES14463

NPC1 rabbit pAb

Sizes: 50μL, 100μL

Catalogue Numbers: ES14463-50, ES14463-100

Citations, Manuals and MSDS Available upon request.

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],

Source: Rabbit

Applications: WB

Dilution: WB 1: 500-2000

Reactivity: Human; Mouse

Immunogen: Synthesized peptide derived from human NPC1 AA range: 1073-1123

Storage and Stability: -20°C/1 year

Clonality: Polyclonal

Isotype: IgG

Concentration: 1 mg/ml

Human Gene ID: 4864

Human SWISS Prot NO: O15118

Subcellular Location: Late endosome membrane; Multi-pass membrane protein. Lysosome membrane; Multi-pass membrane protein.

Research Use Only