Catalogue number: | PA1723 |
Price: | $200.00 |
Reactivities: | Human |
Applications: | Immunocytochemistry, Immunohistochemistry, Western Blot |
Size: | 100ug/vial |
Gene: | ABCB6 |
Swiss prot: | Q9NP58 |
Form: | Lyophilized |
Format: | Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg Thimerosal, 0.05mg NaN3. |
Storage temp: | At -20 degree C for one year. After reconstitution, at 4 degree C for one month. It can also be aliquotted and stored frozen at -20 degree C for a longer time.Avoid repeated freezing and thawing. |
Scientific background: | 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. |
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. 2.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. 3.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. |
Additional info: | A synthetic peptide corresponding to a sequence at the C-terminal of human ABCB6. |