NPC1 Antibody

Catalog Number:

24900

Amount:

100μg

Price:

$319

Swiss-Prot No:

Swiss-Prot:O15118
Gene ID:4864

Form of Antibody:

Supplied in PBS containing 0.02% sodium azide.

Storage/Stability:

Immunogen:

Raised against a 16 amino acid peptide from near the carboxy terminus of human NPC1.

Purification:

Affinity chromatography purified via peptide column

Specificity/Sensitivity:

Applications:

ELISA WB IHC

Background:

Mutations in the Niemann-Pick disease type C1 (NPC1) gene result in a fatal progressive neurodegenerative disorder characterized by an abnormal sequestration of lipids including cholesterol and glycosphingolipids. The NPC1 protein is 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. NPC1 transports low-density lipoproteins to late endosomal/lysosomal compartments where they are hydrolized and released as free cholesterol. NPC1, in addition to FTO, MC4R, and PTER has recently been shown to be a new risk loci for early-onset and morbid adult obesity in European populations. This anti-NPC1 antibody will not cross-react to NPC2, another gene whose defects also result in Niemann-Pick type C disease.

References:

appl_detail:

Datasheet PDF