Rabbit anti-BCKDHA Antibody Affinity Purified
Catalog #
BCKDHA
Human
IP
Rabbit
Polyclonal
Whole IgG
Between 75 and 125
IgG
Unconjugated
Antigen Affinity Purified
Product Details
Human
Human
2 - 8 °C
1 year from date of receipt
BCKDHA (branched chain keto acid dehydrogenase E1, alpha polypeptide) is a component of the branched-chain alpha-keto acid (BCAA) dehydrogenase (BCKD) complex. The BCKD complex is an inner 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). Mutations in this gene result in maple syrup urine disease, type IA [taken from NCBI Entrez Gene (Gene ID: 593)].
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
Alternate Names
2-oxoisovalerate dehydrogenase (lipoamide); 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial; BCKDE1A; BCKDH E1-alpha; branched chain keto acid dehydrogenase E1 alpha protein; branched-chain alpha-keto acid dehydrogenase E1 component alpha chain; MSU; MSUD1; OVD1A
Applications