NPC1

BT-AP11562

Rabbit

IgG

100ul,50ul,20ul

Polyclonal

Synthesized peptide derived from human NPC1

The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.

1 mg/ml

Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

-20°C for 1 year

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.

Human: O15118; Mouse: O35604;

140580

Human: 4864

NPC1

Cardiovascular; Metabolism; Neuroscience; Signal transduction

NPC1