ABCB7 Polyclonal Antibody
Ucallm_WP10882-50
Price range: $268.00 through $328.00
Product details
Background:
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
Specifications
| Size | 50μL/100μL |
| Target | ABCB7 |
| Reactivity | Human, Mouse, Rat |
| Applications | WB, IHC, IF, ELISA |
| MW(Calculated) | |
| MW(Observed) | Human:83kD,Mouse/Rat:100kD |
| Host Species | Rabbit |
| Isotype | IgG |
| Conjugate/Modification | Unmodified |
| Recommended Dilution Ratio | WB 1:500-1:2000; IHC 1:100-1:300; ELISA 1:40000; IF 1:50-200 / Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0 (Cat#WT1004) |
| Formulation | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. |
| Storage | -15°C to -25°C/1 year(Do not lower than -25°C) |
| Concentration | 1 mg/ml |
| Clonality | Polyclonal |
| Clone Number | |
| 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. |
| Gene Name | ABCB7 |
| Protein Name | ATP-binding cassette sub-family B member 7 mitochondrial |
| Other Name | ABCB7; / ABC7; / ATP-binding cassette sub-family B member 7; / mitochondrial; / ATP-binding cassette transporter 7; / ABC transporter 7 protein |
| Species | Human |
| Gene ID | 22 |
| Uniprot | O75027, |
| Species.1 | Mouse |
| Gene ID.1 | |
| Uniprot.1 | Q61102 |
| Species.2 | |
| Gene ID.2 | |
| Uniprot.2 | |
| Cellular Localization | Mitochondrion inner membrane; Multi-pass membrane protein . |
| Tissue Expression | Human esophagus tumor,Muscle,Placenta,Umbilical cord blood, |
| Signaling_pathway | |
| Research Areas | >>ABC transporters |
| 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 ., |
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