名称：NPC1

别名：Niemann-Pick C1 protein, NPC1

Targets：FDA approved drug target related

描述

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.

Specification

Entrez GeneID：4864

Swissprot：O15118

WB Predicted band size：142.2kDa

Host/Isotype：Rabbit IgG

Antibody Type：Primary antibody

Storage：Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.

Species Reactivity：Human, Mouse

免疫原：This NPC1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 591-620 amino acids from the Central region of human NPC1.

Formulation：Purified antibody in PBS with 0.05% sodium azide.

Application

Application：WB,IHC-P,IF

WB：1/1000-1/2000

IHC：1/100-1/500

ICC：1/10-1/50

宿主：Rabbit