|Other Names||2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial, Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain, BCKDE1A, BCKDH E1-alpha, BCKDHA|
|Target/Specificity||The synthetic peptide sequence used to generate the antibody AP6830b was selected from the C-term region of human BCKDHA. A 10 to 100 fold molar excess to antibody is recommended. Precise conditions should be optimized for a particular assay.|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Function||The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).|
|Cellular Location||Mitochondrion matrix.|
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Provided below are standard protocols that you may find useful for product applications.
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). BCKDHA is the alpha subunit of the decarboxylase (E1) component.
Flaschker,N., et.al., J. Inherit. Metab. Dis. 30 (6), 903-909 (2007)
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