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Literature summary for 6.3.4.15 extracted from

  • Dupuis, L.; Leon-del Rio, A.; Leclerc, D.; Campeau, E.; Sweetman, L.; Saudubray, J.M.; Herman, G.; Gibson, K.M.; Gravel, R.A.
    Clustering of mutations in the biotin-binding region of holocarboxylase synthetase in biotin-responsive multiple carboxylase deficiency (1996), Hum. Mol. Genet., 5, 1011-1016.
    View publication on PubMed

Organism

Organism UniProt Comment Textmining
Homo sapiens
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holocarboxylase synthetase catalyzes the biotinylation of the four biotin-dependent carboxylases found in human, mitochondrial propionyl-CoA carboxylase, pyruvate carboxylase, beta-methylcrotonyl-CoA carboxylase, and cytosolic acetyl-CoA carboxylase
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Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
ATP + biotin + apo-[acetyl-CoA:carbon-dioxide ligase (ADP-forming)]
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Homo sapiens AMP + diphosphate + [acetyl-CoA:carbon-dioxide ligase (ADP-forming)]
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