Testing Data
Rabbit ABCB6 Polyclonal Antibody | anti-ABCB6 antibody
ABCB6 Antibody
Testing Data
Testing Data
NCBI and Uniprot Product Information
NCBI Description
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. [provided by RefSeq, Jul 2008]
Uniprot Description
ABCB6: 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). A disorder of eye formation, ranging from small size of a single eye to complete bilateral absence of ocular tissues. Ocular abnormalities like opacities of the cornea and lens, scaring of the retina and choroid, cataract and other abnormalities like cataract may also be present. Ocular colobomas are a set of malformations resulting from abnormal morphogenesis of the optic cup and stalk, and the fusion of the fetal fissure (optic fissure). Belongs to the ABC transporter superfamily. ABCB family. Heavy Metal importer (TC 3.A.1.210) subfamily. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: Membrane protein, multi-pass; Transporter, ABC family; Transporter; Mitochondrial; Membrane protein, integral; Hydrolase
Chromosomal Location of Human Ortholog: 2q36
Cellular Component: mitochondrial envelope; Golgi apparatus; mitochondrial outer membrane; mitochondrion; endoplasmic reticulum; plasma membrane; ATP-binding cassette (ABC) transporter complex; endosome; integral to mitochondrial outer membrane
Molecular Function: efflux transmembrane transporter activity; heme-transporting ATPase activity; heme transporter activity; heme binding; ATP binding
Biological Process: skin development; heme transport; transport; cellular iron ion homeostasis; brain development; transmembrane transport; porphyrin biosynthetic process
Disease: Dyschromatosis Universalis Hereditaria 3; Blood Group, Langereis System; Microphthalmia, Isolated, With Coloboma 7
Research Articles on ABCB6
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Product Notes
The ABCB6 abcb6 (Catalog #AAA2522820) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The ABCB6 Antibody reacts with Human, Mouse, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's ABCB6 can be used in a range of immunoassay formats including, but not limited to, ELISA (EIA), Western Blot (WB). WB: 1:500-1:5000. Researchers should empirically determine the suitability of the ABCB6 abcb6 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "ABCB6, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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