| Product Name | Anti-BCKDHA antibody-RBITC |
|---|---|
| Antibody Type | Primary Antibodies |
| Immunogen | Polypeptide |
| Conjugation | RBITC |
| Clonality | Polyclonal |
|---|---|
| Isotype | IgG |
| Host Species | Rabbit |
| Tested Applications | IFFC |
| Species Reactivity | HumanMouseRat |
| Concentration | 1mg/ml |
| Purification | Protein A |
| Gene Symbol | BCKDHA |
|---|---|
| Gene Synonyms | ODBA BCKDE1A BCKDH E1-alpha BCKDHA |
| Gene Full Name | branched chain keto acid dehydrogenase E1 subunit alpha |
| Gene Summary | The branched-chain alpha-keto acid (BCAA) dehydrogenase (BCKD) complex is an innter mitochondrial enzyme complex that catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. The BCKD complex consists of three catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). This gene encodes the alpha subunit of the decarboxylase (E1) component. Mutations in this gene result in maple syrup urine disease, type IA. Multiple transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Sep 2009] |
| Molecular Weight(MW) | 50kDa |
| Source | Rabbit |
| Cellular Localization | Mitochondrion |
| SwissProt ID | P12694 |
|---|
| Form | Liquid |
|---|---|
| Storage Instructions | Shipped at 4°C. Store at +4°C short term (1-2 weeks). Store at -20°C long term. Avoid freeze / thaw cycle. |
| Storage Buffer | Purified antibody in PBS with 0.05% sodium azide. |
