BCKDHA antibody [HL2434]

GeneTex

BCKDHA antibody [HL2434]

9

WB: 1:500-1:3000. *Optimal dilutions/concentrations should be determined by the researcher.Not tested in other applications.

Western Blot, ImmunoHistoChemistry, ImmunoHistoChemistry Paraffin

Research Use Only

Monoclonal

HL2434

1 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 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). This gene encodes the alpha subunit of the decarboxylase (E1) component. Mutations in this gene result in maple syrup urine disease, type IA. Multiple transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Sep 2009]

Human, Mouse, Rat, Zebra Fish

-20°C or -80°C,2°C to 8°C

12352203