Niemann Pick C1 Rabbit mAb

Catalog Number:

77061

Amount:

100μg

Price:

$318

Swiss-Prot No:

O15118

Form of Antibody:

Storage/Stability:

Immunogen:

A synthesized peptide derived from human Niemann Pick C1.

Purification:

Affinity purification

Specificity/Sensitivity:

Applications:

WB,IHC,IF

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.

References:

appl_detail:

WB 1:500 - 1:2000
IHC 1:50 - 1:200
IF 1:50 - 1:200

Datasheet PDF