BCKDHA Polyclonal Antibody
SKU: E-AB-93313-200
BCKDHA Polyclonal Antibody
| SKU # | E-AB-93313 |
| Reactivity | Human |
| Host | Rabbit |
| Applications | WB, IF |
Product Details
| Isotype | IgG |
| Host | Rabbit |
| Reactivity | Human |
| Applications | WB, IF |
| Clonality | Polyclonal |
| Immunogen | Recombinant fusion protein of human BCKDHA |
| Abbre | BCKDHA |
| Synonyms | 2-oxoisovalerate dehydrogenase subunit alpha, BCKDE1A, MSU, MSUD1, OVD1A, mitochondrial, BCKDHA |
| Swissprot | |
| Calculated MW | 50 kDa |
| Observed MW | Refer to figures |
| Cellular Localization | Mitochondrion matrix. |
| Concentration | 1 mg/mL |
| Buffer | Phosphate buffered solution, pH 7.4, containing 0.05% stabilizer and 50% glycerol. |
| Purification Method | Affinity purification |
| Conjugation | Unconjugated |
| Storage | Store at -20°C Valid for 12 months. Avoid freeze / thaw cycles. |
| Shipping | The product is shipped with ice pack,upon receipt,store it immediately at the temperature recommended. |
Related Reagents
| Applications | Recommended Dilution |
| WB | 1:500-1:2000 |
| IF | 1:50-1:200 |
Background
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.