NPC1 antibody (AA 1022-1278)

Catalog No. ABIN5693129

Product Details anti-NPC1 Antibody

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

Binding Specificity: AA 1022-1278

Reactivity: Human, Mouse, Rat

Host: Rabbit

Clonality: Polyclonal

Conjugate: This NPC1 antibody is un-conjugated

Application: ELISA, Western Blotting (WB)

Cross-Reactivity (Details): No cross reactivity with other proteins.

Characteristics: Rabbit IgG polyclonal antibody for Niemann Pick C1 detection. Tested with WB, Direct ELISA in Human,Mouse,Rat.

Immunogen: E. coli-derived human Niemann Pick C1 recombinant protein (Position: A1022-F1278).

Application Details

Application Notes:

Recommended Detection Systems: Enhanced Chemiluminescent Kit with anti-Rabbit IgG (ABIN921124) for Western blot.

Application Details: Western blot,0.1-0.5 μg/mL
Direct ELISA,0.1-0.5 μg/mL

Restrictions: For Research Use only

Handling

Format: Lyophilized

Reconstitution: Add 0.2 mL of distilled water will yield a concentration of 500 μg/mL.

Buffer: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na₂HPO₄, 0.05 mg NaN₃.

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: At -20°C for one year. After reconstitution, at 4°C for one month.
It can also be aliquotted and stored frozen at -20°C for a longer time. Avoid repeated freezing and thawing.

Target Details for NPC1

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

Alternative Name: NPC1 (NPC1 Products)

Synonyms: NPC antibody, 9130221N23Rik antibody, Gm243 antibody, Cdig2 antibody, im:7149020 antibody, wu:fb53a12 antibody, wu:fc29a12 antibody, A430089E03Rik antibody, C85354 antibody, D18Ertd139e antibody, D18Ertd723e antibody, lcsd antibody, nmf164 antibody, spm antibody, NPC intracellular cholesterol transporter 1 antibody, NPC1 like intracellular cholesterol transporter 1 antibody, Niemann-Pick disease, type C1 antibody, Niemann-Pick C1 protein antibody, NPC1 antibody, Npc1l1 antibody, Npc1 antibody, npc1 antibody, LOC579887 antibody

Background:

Synonyms: Niemann-Pick C1 protein, NPC1

Background: 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.

UniProt: O15118