ITT0046
ITT0046
- Catalog: ITT0046
- Gene/Protein: ABCB7
- Product Description: Immunotag™ ABCB7 Polyclonal Antibody
385.0000
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Antibody Specification | |
Datasheet | |
Target Protein | ABCB7 |
Clonality | Polyclonal |
Storage/Stability | -20°C/1 year |
Application | WB,IHC-p,ELISA |
Recommended Dilution | Western Blot: 1/500 - 1/2000. Immunohistochemistry: 1/100 - 1/300. ELISA: 1/40000. Not yet tested in other applications. |
Concentration | 1 mg/ml |
Reactive Species | Human |
Host Species | Rabbit |
Immunogen | The antiserum was produced against synthesized peptide derived from human ABCB7. AA range:691-740 |
Specificity | ABCB7 Polyclonal Antibody detects endogenous levels of ABCB7 protein. |
Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen |
Form | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
Gene Name | ABCB7 |
Accession No. | O75027 Q61102 |
Alternate Names | ABCB7; ABC7; ATP-binding cassette sub-family B member 7; mitochondrial; ATP-binding cassette transporter 7; ABC transporter 7 protein |
Description | ATP binding cassette subfamily B member 7(ABCB7) Homo sapiens 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 gene encodes a half-transporter involved in the transport of heme from the mitochondria to the cytosol. With iron/sulfur cluster precursors as its substrates, this protein may play a role in metal homeostasis. Mutations in this gene have been associated with mitochondrial iron accumulation and isodicentric (X)(q13) and sideroblastic anemia. Alternatively spliced transcript variants encoding multiple isoforms hav |
Cell Pathway/ Category | ABC transporters, |
Protein Expression | Human esophagus tumor,Muscle,Placenta,Umbilical cord blood, |
Subcellular Localization | mitochondrion,mitochondrial inner membrane,integral component of membrane, |
Protein Function | disease:Defects in ABCB7 are the cause of X-linked sideroblastic anemia with ataxia (ASAT) [MIM:301310]. ASAT is a recessive disorder characterized by an infantile to early childhood onset of nonprogressive cerebellar ataxia and mild anemia with hypochromia and microcytosis.,function:Could be involved in the transport of heme from the mitochondria to the cytosol. Plays a central role in the maturation of cytosolic iron-sulfur (Fe/S) cluster-containing proteins.,similarity:Belongs to the ABC transporter family. Heavy Metal importer (TC 3.A.1.210) subfamily.,similarity:Contains 1 ABC transmembrane type-1 domain.,similarity:Contains 1 ABC transporter domain.,subunit:Homodimer or heterodimer ., |
Usage | For Research Use Only! Not for diagnostic or therapeutic procedures. |