NPC1 Antibody| F42992-0.08ML/F42992-0.4ML/R36320-100UG

NPC1 Antibody| F42992-0.08ML/F42992-0.4ML/R36320-100UG | Gentaur UK, US & Europe Distribution

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.

Format: Antigen affinity purified

Clone: N/A

Host Animal: Rabbit

Clonality: Polyclonal (rabbit origin)

Isotype: Rabbit Ig

Species Reactivity: Human

Predicted Spacies Reactivity: N/A

Application: IF, IHC, WB, ELISA

Application Details: Western blot: 1:1000, Immunofluorescence: 1:10-1:50, IHC (Paraffin): 1:10-1:50

Application Note: Titration of the NPC1 antibody may be required due to differences in protocols and secondary/substrate sensitivity.

Status: Available

Buffer: N/A

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

Purity: Antigen affinity

Immunogen: A portion of amino acids 591-620 from the human protein was used as the immunogen for this NPC1 antibody.

Storage: Aliquot the NPC1 antibody and store frozen at -20oC or colder. Avoid repeated freeze-thaw cycles.

Localization: N/A