| Synonyms | ABC7 |
| Swiss Prot Num | O75027 |
| Immunogen | Synthetic Peptide - KLH conjugated corresponding to Amino acids 733 to 747 of human ABCB7. |
| Product Type | Antibodies |
| Storage | Store the antibody undiluted at 2-8°C for one month or (in aliquots) at -20°C for longer. Dilute only prior to immediate use. Avoid cycles of freezing and thawing. Shelf life: One year from despatch. |
|
| Format | State: Liquid purified IgG fraction. |
| ? | Purification: Protein G Chromatography. |
| ? | Buffer System: Phosphate Buffered Saline PBS containing 0.09% Sodium Azide as preservative. |
|
| Specificity | This antibody recognises ATP-Binding Cassette Sub-Family B. |
| Application | Immunohistochemistry on Paraffin Sections (10 ?g/ml). ELISA (1/1000). |
| Background | ABCB7 is a mitochondrial protein involved in the transport of heme from the mitochondria to the cystol. Defects in ABCB7 are the cause of X-linked sideroblastic anemia with ataxia (ASAT). ASAT is a recessive disorder characterized by mitochondrial iron deposition and excess of protoporphyrin in erythroid cells. ASAT is also characterized by an infantile to early childhood onset of non-progressive cerebellar ataxia and mild anemia with hypochromia and microcytosis. |
