Anti-Niemann Pick C2/NPC2 Picoband® Antibody

SKU: A01582-3

Size: 100 μg

Storage: Store 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 freeze-thaw cycles.

Form: Lyophilized

Reactivity: Human,Mouse

Applications: ELISA,Flow Cytometry,IF,IHC,ICC,WB

Application Details: Western blot, 0.25-0.5μg/ml, Human
Immunohistochemistry (Paraffin-embedded Section), 0.5-1μg/ml, Human, Mouse
Immunocytochemistry/Immunofluorescence, 2μg/ml, Human
Flow Cytometry (Fixed), 1-3μg/1x10⁶ cells, Human
ELISA, 0.1-0.5μg/ml, -

Gene Name: NPC2

Specificity: No cross reactivity with other proteins.

Background: Epididymal secretory protein E1 (also called NPC intracellular cholesterol transporter 2, or NPC2) is a protein associated with Niemann-Pick disease, type C. It is mapped to 14q24.3. This gene encodes a protein containing a lipid recognition domain. The encoded protein may function in regulating the transport of cholesterol through the late endosomal/lysosomal system. Mutations in this gene have been associated with Niemann-Pick disease, type C2 and frontal lobe atrophy.

Immunogen: E.coli-derived human Niemann Pick C2/NPC2 recombinant protein (Position: E20-L151).

Clonality: Polyclonal

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

Purification: Immunogen affinity purified.

Reconstitution: Add 0.2ml of distilled water will yield a concentration of 500ug/ml.

Reference: 1. Blom, T. S., Linder, M. D., Snow, K., Pihko, H., Hess, M. W., Jokitalo, E., Veckman, V., Syvanen, A.-C., Ikonen, E. Defective endocytic trafficking of NPC1 and NPC2 underlying infantile Niemann-Pick type C disease. Hum. Molec. Genet. 12: 257-272, 2003. 2. Sleat, D. E., Wiseman, J. A., El-Banna, M., Price, S. M., Verot, L., Shen, M. M., Tint, G. S., Vanier, M. T., Walkley, S. U., Lobel, P. Genetic evidence for nonredundant functional cooperativity between NPC1 and NPC2 in lipid transport. Proc. Nat. Acad. Sci. 101: 5886-5891, 2004. 3. Verot, L., Chikh, K., Freydiere, E., Honore, R., Vanier, M. T., Millat, G. Niemann-Pick C disease: functional characterization of three NPC2 mutations and clinical and molecular update on patients with NPC2. Clin. Genet. 71: 320-330, 2007.

Uniprot ID: P61916

Host: Rabbit

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

Conjugate: This is the unconjugated antibody. It is also available with 10 conjugation options, including Biotin, Cy3, Fluoro488, Fluoro550, Fluoro594, FITC, HRP, APC, PE, and Fluoro647. A carrier-free (BSA-free) version is also available. Additional charges may apply for some options. Please contact us for a quote

Cross Reactivity: No cross-reactivity with other proteins.

Isotype: Rabbit IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 19 kDa

Calculated Molecular Weight:

Gene ID: 10577

Protein Name: NPC intracellular cholesterol transporter 2

Gene Full Name: NPC intracellular cholesterol transporter 2

Synonyms: HE1; Niemann Pick disease; type C2; NPC2