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Literature summary extracted from

  • Tsai, H.Y.; Wu, S.C.; Li, J.C.; Chen, Y.M.; Chan, C.C.; Chen, C.H.
    Loss of the Drosophila branched-chain alpha-keto acid dehydrogenase complex (BCKDH) results in neuronal dysfunction (2020), Dis. Model. Mech., 13, dmm044750 .
    View publication on PubMed

Application

EC Number Application Comment Organism
2.3.1.168 medicine creation of a Drosophila model of maple syrup urine disease MSUD by knocking out the DBT gene, an ortholog of the human gene encoding the dihydrolipoamide branched chain transacylase (DBT) subunit of BCKDH. The homozygous DBT mutant larvae recapitulate an array of MSUD phenotypes, including aberrant branched-chain amino acid accumulation, developmental defects, poor mobile behavior and disrupted L-glutamate homeostasis. The DBT mutation causes neuronal apoptosis during the developmental progression of larval brains and severe impairment of retinal rhabdomeres. The DBT mutant shows elevated oxidative stress and higher lipid peroxidation accumulation in the larval brain. When the DBT mutants are administrated with antidiabetic drug metformin, the aberrances in branched-chain amino acid levels and motor behavior are ameliorated Drosophila melanogaster

Organism

EC Number Organism UniProt Comment Textmining
2.3.1.168 Drosophila melanogaster
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Synonyms

EC Number Synonyms Comment Organism
2.3.1.168 DBT
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 Drosophila melanogaster
2.3.1.168 dihydrolipoamide branched chain transacylase
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 Drosophila melanogaster