84-4903-66　BCKDHA (2-Oxoisovalerate Dehydrogenase Subunit Alpha, Mitochondrial, Branched Chain Keto Acid Dehydrogenase E1, Alpha Polypeptide) 100ul　476156

特徴

• The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO2.
• It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).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].
• Applications:Suitable for use in Immunohistochemistry, Western Blot.
• Other applications not tested.
• Recommended Dilution:Optimal dilutions to be determined by the researcher.
• Storage and Stability:May be stored at 4°C for short-term only.
• Aliquot to avoid repeated freezing and thawing.
• Store at -20°C.
• Aliquots are stable for 12 months after receipt.
• For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.