Cet anticorps anti-BCKDHA Monoclonal Lapin (Clone BLR060F) (ABIN7449282) détecte spécifiquement BCKDHA dans WB.
L’anticorps est réactif avec des échantillons de Humain et Souris.
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)].