NPC1 antibody (AA 34-174)

Catalog No. ABIN1845969

Product Details anti-NPC1 Antibody

Target: NPC1 (Niemann-Pick Disease, Type C1 (NPC1))

Binding Specificity: AA 34-174

Reactivity: Human

Host: Mouse

Clonality: Monoclonal

Conjugate: This NPC1 antibody is un-conjugated

Application: ELISA, Western Blotting (WB), Immunohistochemistry (IHC), Flow Cytometry (FACS)

Purification: Purified

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

Isotype: IgG1

Application Details

Restrictions: For Research Use only

Handling

Buffer: Purified antibody in PBS with 0.05% sodium azide and 0.5% protein stabilizer.

Preservative: Sodium azide

Precaution of Use: This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Storage: -20 °C

Storage Comment: Aliquot and store at -20 °C.

Target Details for NPC1

Target: NPC1 (Niemann-Pick Disease, Type C1 (NPC1))

Alternative Name: NPC1 (NPC1 Products)

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.

Molecular Weight: 142.2 kDa