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BCKDHA Antibody (C-term) Blocking Peptide

Synthetic peptide

     
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Product Information
Primary Accession P12694
Clone Names 90121078
Additional Information
Gene ID 593
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 Peptides are lyophilized in a solid powder format. Peptides can be reconstituted in solution using the appropriate buffer as needed.
StorageMaintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.
PrecautionsThis product is for research use only. Not for use in diagnostic or therapeutic procedures.
Protein Information
Name BCKDHA (HGNC:986)
Function Together with BCKDHB forms the heterotetrameric E1 subunit of the mitochondrial branched-chain alpha-ketoacid dehydrogenase (BCKD) complex. The BCKD complex catalyzes the multi-step oxidative decarboxylation of alpha-ketoacids derived from the branched-chain amino-acids valine, leucine and isoleucine producing CO2 and acyl-CoA which is subsequently utilized to produce energy. The E1 subunit catalyzes the first step with the decarboxylation of the alpha-ketoacid forming an enzyme-product intermediate. A reductive acylation mediated by the lipoylamide cofactor of E2 extracts the acyl group from the E1 active site for the next step of the reaction.
Cellular Location Mitochondrion matrix
Research Areas
Citations (0)
citation

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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). BCKDHA is the alpha subunit of the decarboxylase (E1) component.

References

Flaschker,N., et.al., J. Inherit. Metab. Dis. 30 (6), 903-909 (2007)

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$ 277.78
Cat# BP6830b
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Availability: 2 weeks
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