NPC1 Antibody

Western blot: 1:1000,Immunofluorescence: 1:10-1:50,IHC (Paraffin): 1:10-1:50

Titration of the NPC1 antibody may be required due to differences in protocols and secondary/substrate sensitivity.

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.

Antigen affinity purified

In 1X PBS, pH 7.4, with 0.09% sodium azide

A portion of amino acids 591-620 from the human protein was used as the immunogen for this NPC1 antibody.

This NPC1 antibody is available for research use only.

Antigen affinity

Human

O15118