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GeneBio Systems

Anti-Niemann Pick C2/NPC2 Antibody Picoband®

Anti-Niemann Pick C2/NPC2 Antibody Picoband®

SKU:A01582-2

Regular price €506,95 EUR
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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

Applications: IHC,WB

Application Details: Western blot,0.1-0.5μg/ml, Human
Immunohistochemistry (Paraffin-embedded Section),2-5μg/ml, Human

Gene Name: NPC2

Specificity: No cross reactivity with other proteins.

Background: NPC2 is a protein associated with Niemann-Pick disease, type C. This gene is mapped to chromosome 14q24.3. It 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: A synthetic peptide corresponding to a sequence at the C-terminus of human Niemann Pick C2, which shares 79.4% and 76.5% amino acid (aa) sequence identity with mouse and rat Niemann Pick C2, respectively.

Clonality: Polyclonal

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

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. Klunemann, H. H., Elleder, M., Kaminski, W. E., Snow, K., Peyser, J. M., O'Brien, J. F., Munoz, D., Schmitz, G., Klein, H. E., Pendlebury, W. W. Frontal lobe atrophy due to a mutation in the cholesterol binding protein HE1/NPC2. Ann. Neurol. 52: 743-749, 2002.

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: 16 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

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