Product Data | |
Description | Recombinant protein of human dihydrolipoamide branched chain transacylase E2 (DBT), nuclear gene encoding mitochondrial protein |
Species | Human |
Expression Host | HEK293T |
Expression cDNA Clone or AA Sequence | Recombinant protein was produced with TrueORF clone, RC201998. Click on the TrueORF clone link to view cDNA and protein sequences. |
Tag | C-Myc/DDK |
Predicted MW | 46.4 kDa |
Concentration | >50 ug/mL as determined by microplate BCA method |
Purity | > 80% as determined by SDS-PAGE and Coomassie blue staining |
Buffer | 25 mM Tris.HCl, pH 7.3, 100 mM glycine, 10% glycerol |
Reference Data | |
Locus ID | 1629 |
Refseq Size | 10831 |
Cytogenetics | 1p21.2 |
Refseq ORF | 1446 |
Synonyms | BCATE2; BCKAD-E2; BCKADE2; BCOADC-E2; E2; E2B |
Summary | The 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, Jul 2008] |
Protein Families | Druggable Genome |
Protein Pathways | Metabolic pathways, Valine, leucine and isoleucine degradation |
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