- Catalog number
- PA1723-Dylight550
- Size / count
- 100 µg
- Dry ice
- No
- Hazardous
- No
- Discontinued
- Yes
- 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.
- Synonyms
- ATP-binding cassette sub-family B member 6, mitochondrial;Mitochondrial ABC transporter 3;Mt-ABC transporter 3;P-glycoprotein-related protein;Ubiquitously-expressed mammalian ABC half transporter;ABCB6;MTABC3, PRP, UMAT;
- Gene Name
- ATP-binding cassette sub-family B member 6, mitochondrial
- UniProt
- Q9NP58
- Host
- Rabbit
- Reactivity
- Human
- Cross Reactivity
- No cross-reactivity with other proteins
- Immunogen
- A synthetic peptide corresponding to a sequence at the C-terminus of human ABCB6.
- Clonality
- Polyclonal
- Tissue Specificity
- Widely expressed. High expression is detected in the retinal epithelium. .
- Applications
- IHC,ICC,WB
- Purification
- Immunogen affinity purified.
- Concentration
- Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.
- Form
- Lyophilized
- Reconstitution
- Add 0.2ml of distilled water will yield a concentration of 500ug/ml.
- Function
- Binds heme and porphyrins and functions in their ATP- dependent uptake into the mitochondria. Plays a crucial role in heme synthesis. .
- Components
- Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg Thimerosal, 0.05mg NaN3.
- References & Citations
- 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.
- Storage Conditions
- 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.
- Calculated Molecular Weight
- 93886 MW
- Observed Molecular Weight
- 94 kDa
- Fragment
- Rabbit IgG
- Specificity
- No cross reactivity with other proteins.
- Applications Notes
- Immunocytochemistry , 0.5-1μg/ml, Human, -<br>Immunohistochemistry (Paraffin-embedded Section), 0.5-1μg/ml, Human, By Heat<br>Western blot, 0.1-0.5μg/ml, Human<br>
- Subcellular Location
- Cell membrane. Mitochondrion outer membrane; Multi-pass membrane protein. Endoplasmic reticulum. Golgi apparatus. Endosome . localized to the endosome- like compartement and dendrite tips.
- Sequence Similarities
- Belongs to the ABC transporter superfamily. ABCB family. Heavy Metal importer (TC 3.A.1.210) subfamily.
- Protein Name
- ATP-binding cassette sub-family B member 6, mitochondrial