NPC1 Antibody / Niemann Pick C1 | RQ4395

NPC1 Antibody / Niemann Pick C1 | RQ4395 | Gentaur US, UK & Europe Disrtribition

Family: Primary antibody

Formulation: 0.5mg/ml if reconstituted with 0.2ml sterile DI water

Format: Antigen affinity purified

Clone: N/A

Host Animal: Rabbit

Clonality: Polyclonal (rabbit origin)

Species Reactivity: Human, Mouse, Rat

Application: WB, ELISA

Buffer: Lyophilized from 1X PBS with 2% Trehalose and 0.025% sodium azide

Limitation: This NPC1 antibody is available for research use only.

Purity: Antigen affinity purified

Description: Niemann-Pick disease, type C1 (NPC1) is a membrane protein that mediates intracellular cholesterol trafficking in mammals. In humans it is encoded by the NPC1 gene (chromosome location 18q11). 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.

Immunogen: A human partial recombinant protein corresponding to amino acids A1022-F1278 was used as the immunogen for the NPC1 antibody.

Storage: After reconstitution, the NPC1 antibody can be stored for up to one month at 4oC. For long-term, aliquot and store at -20 °C. Avoid repeated freezing and thawing.