免疫原
Recombinant protein (or fragment).This information is considered to be commercially
sensitive.
纯化工艺
Affinity purification
储存
Store at -20℃. Avoid freeze / thaw cycles.
Buffer: PBS with 0.02% sodium azide,50% glycerol,pH7.3.
其他名称
MSU; MSUD1; OVD1A; BCKDE1A; BCKDHA
背景
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.
