ABCB6 (Mitochondrial ABC transporter 3)

ABCB6, ATP-binding cassette sub-family B member 6, mitochondrialis a protein that in humans is encoded by the ABCB6 gene. The membrane-associated protein encoded by this gene is a member of the superfamily of ATP-binding cassette (ABC) transporters. ABC proteins transport various molecules across extra- and intra-cellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). This protein is a member of the MDR/TAP subfamily. Members of the MDR/TAP subfamily are involved in multidrug resistance as well as antigen presentation. This half-transporter likely plays a role in mitochondrial function. Localized to 2q26, this gene is considered a candidate gene for lethal neonatal metabolic syndrome, a disorder of mitochondrial function.\n\nUniProt Number:\nQ9NP58\n\nGene ID:\nABCB6\n\nApplications:\nSuitable for use in Western Blot, Immunohistochemistry (Paraffin) and Immunocytochemistry.\n\nRecommended Dilution:\nOptimal dilutions to be determined by the researcher.\n\nStorage and Handling:\nStore at -20?C for one year. After reconstitution, store at 4?C for one month. Can also be aliquoted and stored frozen at -20?C for long term.\nAvoid repeated freezing and thawing. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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SPECIFICATIONS

Catalog Number

144103

Size

100ug

Applications

ICC, IHC, WB

Hosts

Rabbit

Reactivities

Hum

Form

Supplied as a lyophilized powder. Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg Thimerosal, 0.05mg NaN3. Reconstitution: Add 0.2ml of distilled water will yield a concentration of 500ug/ml.

P Type

Pab

Purity

Purified by immunoaffinity chromatography.

Isotype

IgG

References

1.Helias, V., Saison, C., Ballif, B. A., Peyrard, T., Takahashi, J., Takahashi, H., Tanaka, M., Deybach, J.-C., Puy, H., Le Gall, M., Sureau, C., Pham, B.-N., Le Pennec, P.-Y., Tani, Y., Cartron, J.-P., Arnaud, L.ABCB6 is dispensable for erythropoiesis and specifies the new blood group system Langereis.Nature Genet. 44: 170-173, 2012.\n2.Krishnamurthy, P. C., Du, G., Fukuda, Y., Sun, D., Sampath, J., Mercer, K. E., Wang, J., Sosa-Pineda, B., Murti, K. G., Schuetz, J. D.Identification of a mammalian mitochondrial porphyrin transporter.Nature 443: 586-589, 2006.\n3.Wang, L., He, F., Bu, J., Liu, X., Du, W., Dong, J., Cooney, J. D., Dubey, S. K., Shi, Y., Gong, B., Li, J., McBride, P. E., and 11 others.ABCB6 mutations cause ocular coloboma.Am. J. Hum. Genet. 90: 40-48, 2012.\n\n

Additional Info

Recognizes human ABCB6. No crossreactivity with other proteins.