Overview
- SupplierGeneTex
- Product NameDBT antibody
- Delivery Days Customer9
- CertificationResearch Use Only
- Concentration0.3 mg/ml
- IsotypeIgG
- Scientific DescriptionThe branched-chain alpha-keto acid dehydrogenase complex (BCKD) is an inner-mitochondrial enzyme complex involved in the breakdown of the branched-chain amino acids isoleucine, leucine, and valine. The BCKD complex is thought to be composed of a core of 24 transacylase (E2) subunits, and associated decarboxylase (E1), dehydrogenase (E3), and regulatory subunits. This gene encodes the transacylase (E2) subunit. Mutations in this gene result in maple syrup urine disease, type 2. Alternatively spliced transcript variants have been described, but their biological validity has not been determined. [provided by RefSeq]
- Storage Instruction2°C to 8°C,-20°C
- UNSPSC12352203
References
- Functional Assessment of Lipoyltransferase-1 Deficiency in Cells, Mice, and Humans. Ni M et al., 2019 Apr 30, Cell RepRead more