BCKDHA antibody [C1C3]

GeneTex

BCKDHA antibody [C1C3]

9

WB: 1:500-1:3000. ICC/IF: 1:100-1:1000. IHC-P: 1:100-1:1000. *Optimal dilutions/concentrations should be determined by the researcher.Not tested in other applications.

ImmunoFluorescence, Western Blot, ImmunoCytoChemistry, ImmunoHistoChemistry, ImmunoHistoChemistry Paraffin

Research Use Only

Polyclonal

0.31 mg/ml

Unconjugated

BCKDHA

branched chain keto acid dehydrogenase E1 subunit alpha

BCKDE1A, MSU, MSUD1, MSUD1A, OVD1A, 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial, 2-oxoisovalerate dehydrogenase (lipoamide), BCKDH E1-alpha, branched chain keto acid dehydrogenase E1 alpha protein, branched chain keto acid dehydrogenase E1, alpha polypeptide, branched-chain alpha-keto acid dehydrogenase E1 component alpha chain

Rabbit

IgG

2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial

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.[supplied by OMIM]

Human, Mouse, Rat

-20°C or -80°C,2°C to 8°C

12352203