NPC1 antibody (AA 34-174)

Catalog No. ABIN1724840

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, Immunohistochemistry (IHC), Flow Cytometry (FACS)

Purpose: NPC1 Antibody

Purification: Purified antibody

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

Clone: 8D10G3

Isotype: IgG1

Application Details

Application Notes:

ELISA: 1/10000

FCM: 1/200 - 1/400

Restrictions: For Research Use only

Handling

Format: Liquid

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

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: 4 °C,-20 °C

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

References for anti-NPC1 antibody (ABIN1724840)

Morales, Amigo, Balboa, Acuña, Castro, Molina, Miquel, Nervi, Rigotti, Zanlungo: "Deficiency of Niemann-Pick C1 protein protects against diet-induced gallstone formation in mice." in: Liver international : official journal of the International Association for the Study of the Liver, Vol. 30, Issue 6, pp. 887-97, (2010) (PubMed).

Xu, Zhou, Xia, Xia, Chen, Duan, Luo: "Defects of synaptic vesicle turnover at excitatory and inhibitory synapses in Niemann-Pick C1-deficient neurons." in: Neuroscience, Vol. 167, Issue 3, pp. 608-20, (2010) (PubMed).

Target Details for NPC1

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

Alternative Name: NPC1 (NPC1 Products)

Background:

Description: 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.

Aliases: NPC

Molecular Weight: 142.2kDa

Gene ID: 4864

HGNC: 4864

UniProt: O15118