NPC1 Monoclonal Antibody

SKU: BT-MCA4824

Size:100μL

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.

Research_area:Cardiovascular, Metabolism, Neuroscience, Signal transduction

Target_protein:NPC1

applications:IHC, FCM

Reactivity:Human

Clonality:Monoclonal

Clone ID:8D10G3

Host:Mouse

Isotype:Mouse IgG1

Gene Symbol/ Name:NPC1

Immunogen:Purified recombinant fragment of human NPC1 (AA: 34-174) expressed in E. Coli.

Storage:4°C|-20°C for long term storage

Purification:

Formulation:Purified antibody in PBS with 0.05% sodium azide

Concentration:

Molecular weight(Da):142.2kDa

UniProt accession:O15118

Synonyms:NPC

GeneID:4864