Code
OSA00215W
$240USD
ID Tag
Rb1960-091212-WS
Immunogen
A synthetic peptide from aa region 200-250 of human ABCB6 conjugated to an immunogenic carrier protein was used as the antigen.
Accession
Also known
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, MTABC3, PRP, UMAT
Target
FUNCTION: Binds heme and porphyrins and functions in their ATP-dependent uptake into the mitochondria. Plays a crucial role in heme synthesis.
Defects in ABCB6 are the cause of microphthalmia, isolated, with coloboma, type 7 (MCOPCB7).
Subcellular location: Cell membrane. Mitochondrion outer membrane; Multi-pass membrane protein. Endoplasmic reticulum. Golgi apparatus
Tissue specificity: Widely expressed. High expression is detected in the retinal epithelium.
Storage
Maintain the lyophilised/reconstituted antibodies frozen at -20°C for long term storage and refrigerated at 2-8°C for a shorter term. When reconstituting, glycerol (1:1) may be added for an additional stability. Avoid freeze and thaw cycles.
Expiry Date
12 months after reconstitution
Shipping
This item will be shipped to you at ambient temperature in a lyophilised form.
References
1. Mitsuhashi N, et al. J. Biol. Chem. 275:17536-17540(2000)
2. Helias V, et al. Nat. Genet. 44:170-173(2012)
Limitation
For research use only
Related
products
Code
OSA00215W
$240USD
Unit size
100 µl
Conjugate
Unconjugated antibody
Host
NZ white rabbit
Purity
Whole serum
Clonality
Polyclonal
Isotype
Polyclonal, whole serum
Applications
IHC, WB. A dilution of 1 : 300 to 1 : 2000 is recommended. The optimal dilution should be determined by the end user. Not yet tested in other applications.
Specificity
Specific for ABCB6.
Spcs X-react.
Human. Other species not yet tested.
Format
Lyophilised
Reconstitution
Reconstitute in 100 µl of sterile water. Centrifuge to remove any insoluble material.