| Field | Specification |
|---|---|
| Mfr No | |
| Enzyme Type | |
| Expression System | |
| Form | Liquid |
| Formulation | |
| Product Type | |
| Purity | |
| Source | Escherichia Coli. |
| Species | |
| Storage | |
| Target |
Product Description
Branched Chain keto Acid Dehydrogenase E1 Alpha Human Recombinant
Overview
Branched-chain ?-keto acid dehydrogenase E1 component ? chain (BCKDHA) is a member of the BCKDHA family. The BCKD (branched-chain alpha-keto acid dehydrogenase) complex is an inner mitochondrial enzyme complex which catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. This complex consists of 3 catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). Mutations in the BCKDHA gene result in maple syrup urine disease, type IA.
Specifications
| Purity | Greater than 80.0% as determined by SDS-PAGE. |
|---|---|
| Formulation | The BCKDHA solution (0.25 mg/ml) contains 20mM Tris-HCl buffer (pH 8.0), 5mM DTT, 30% glycerol and 0.2M NaCl. |
| Storage | Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles. |
| Expression System | Escherichia Coli. |