NPC Intracellular Cholesterol Transporter 1 (NPC1) Antibody | abx015944

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.

Target: NPC1
Clonality: Monoclonal
Reactivity: Human
Tested Applications: ELISA, WB, IHC, FCM
Host: Mouse
Recommended dilutions: ELISA: 1/10000, WB: 1/500 - 1/2000, IHC: 1/200 - 1/1000, FCM: 1/200 - 1/400. Optimal dilutions/concentrations should be determined by the end user.
Conjugation: Unconjugated
Immunogen: Purified recombinant fragment of human NPC1 (AA: 34-174) expressed in E. coli.
Isotype: IgG1
Form: Liquid
Purification: Purified from ascites by Protein G chromatography.
Storage: Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles.