Anti-Niemann Pick C1/NPC1 Antibody Picoband®

SKU: A00428-3

Size: 100 μg

Storage: At -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freezing and thawing.

Form: Lyophilized

Reactivity: Human,Mouse,Rat

Applications: WB,Flow Cytometry

Application Details: Western blot, 0.25-0.5 μg/ml, Human, Mouse, Rat
Flow Cytometry (Fixed), 1-3 μg/1x10⁶ cells, Human

Gene Name: NPC1

Specificity:

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-absorbance 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 synthetic peptide corresponding to a sequence in the middle region of human Niemann Pick C1/NPC1.

Clonality: Polyclonal

Contents: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

Purification: Immunogen affinity purified.

Reconstitution: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Reference: 1. Entrez Gene: NPC1 Niemann-Pick disease, type C1. 2. Carstea ED, Polymeropoulos MH, Parker CC, Detera-Wadleigh SD, O'Neill RR, Patterson MC, Goldin E, Xiao H, Straub RE, Vanier MT (March 1993). Linkage of Niemann-Pick disease type C to human chromosome 18. Proc. Natl. Acad. Sci. U.S.A. 90 (5): 2002–4.

Uniprot ID: O15118

Host: Rabbit

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate:

Cross Reactivity: No cross-reactivity with other proteins.

Isotype: Rabbit IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 180 kDa

Calculated Molecular Weight:

Gene ID: 4864

Protein Name: NPC intracellular cholesterol transporter 1

Gene Full Name: NPC intracellular cholesterol transporter 1

Synonyms: Niemann Pick C1 protein; Niemann Pick disease; type C1; NPC1