BCKDHA Antibody

The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO2. It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3) .The BCKDHA gene encodes the E1-alpha subunit of the branched-chain alpha-keto acid (BCAA) dehydrogenase complex (BCKD; EC 1.2.4.4), an inner-mitochondrial enzyme complex that catalyzes the oxidative decarboxylation of the branched-chain alpha-ketoacids derived from isoleucine, leucine, and valine. This reaction is the second major step in the catabolism of the branched-chain amino acids (Wynn et al., 1998 [PubMed 9582350]) . The BCKD complex consists of 3 catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a homo-24-meric dihydrolipoyl transacylase (E2; MIM 248610), and a homodimeric dihydrolipoamide dehydrogenase (E3; MIM 238331) . E1 is a thiamine pyrophosphate (TPP) -dependent enzyme. The reaction is irreversible and constitutes the first committed step in BCAA oxidation. The BCKDHB gene (MIM 248611) encodes the beta subunit of E1. The complex also contains 2 regulatory enzymes, a kinase and a phosphorylase.

593

P12694

NP_000700

Human, Mouse, Rat

Antibody produced in rabbits immunized with a synthetic peptide corresponding a region of human BCKDHA.

Unconjugated

Primary Antibodies

Signal Transduction

Antibody is purified by peptide affinity chromatography method.

Cat. No. 1219 - MCF7 Cell Lysate

Batch dependent

Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.

None

Blue Ice

For short periods of storage (days) store at 4˚ C. For longer periods of storage, store BCKDHA antibody at -20˚ C. As with any antibody avoid repeat freeze-thaw cycles.

50 kDa

BCKDHA antibody can be used for detection of BCKDHA by ELISA at 1:312500. BCKDHA antibody can be used for detection of BCKDHA by western blot at 1 μ g/mL, and HRP conjugated secondary antibody should be diluted 1:50,000 - 100,000.

BCKDHA

Branched chain keto acid dehydrogenase E1, alpha polypeptide

Homo sapiens

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

BCKDHA, FLJ45695, MSU, MSUD1, OVD1A, BCKDE1A

ELISA, WB

11386135