2.3.1.168: dihydrolipoyllysine-residue (2-methylpropanoyl)transferase
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For detailed information about dihydrolipoyllysine-residue (2-methylpropanoyl)transferase, go to the full flat file.
Word Map on EC 2.3.1.168
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2.3.1.168
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branched-chain
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bcaas
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merits
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protein-restricted
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retinal
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urine
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metformin
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women
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maple
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l-glutamate
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syrup
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agriculture
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synthesis
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medicine
- 2.3.1.168
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branched-chain
-
bcaas
-
merits
-
protein-restricted
- retinal
- urine
- metformin
- women
-
maple
- l-glutamate
- syrup
- agriculture
- synthesis
- medicine
Reaction
Synonyms
Bkd, BKDH, DBT, dihydrolipoamide branched chain transacylase, dihydrolipoyl transacetylase, DLD, E2, E2p, More
ECTree
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Application
Application on EC 2.3.1.168 - dihydrolipoyllysine-residue (2-methylpropanoyl)transferase
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agriculture
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mutation of promoter binding protein SPL16/GW8 leads to upregulation of pyruvate kinase, pyruvate dehydrogenase E1, dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex, acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial pyruvate carrier, 4-hydroxyphenylpyruvate dioxygenase, and dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex. SPL16 mutations have the potential to boost the grain yield of rice
medicine
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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
synthesis
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functional expression of the branched chain alpha-keto acid dehydrogenase complex in Escherichia coli by independent and selective optimization of individual subunit genes of the complex. Codon optimization significantly improves the expression of complex component proteins BkdH and LpdA1 but expression of dehydrogenase E1 alpha subunit BkdF and dehydrogenase E1 beta subunit BkdG depends on coexpression of the BkdH gene. The optimized branched chain alpha-keto acid dehydrogenase complex supplies sufficient short branched-chain acyl-CoA to synthesize phlorisovalerophenone