Niemann Pick C1 Antibody / NPC1

Western blot: 0.5-1ug/ml, Flow cytometry: 1-3ug/million cells

Optimal dilution of the Niemann Pick C1 antibody should be determined by the researcher.

Lyophilized from 1X PBS with 2% Trehalose

Niemann-Pick disease, type C1 (NPC1) is a membrane protein that mediates intracellular cholesterol trafficking in mammals. In humans it is encoded by the NPC1 gene (chromosome location 18q11). 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-absorbance 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.

Antigen affinity purified

0.5mg/ml if reconstituted with 0.2ml sterile DI water

Amino acids YHTVLQTSADFIDALKKARLI were used as the immunogen for the Niemann Pick C1 antibody.

This Niemann Pick C1 antibody is available for research use only.

Antigen affinity purified

O15118