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Literature summary for 6.3.4.10 extracted from

  • Nyhan, W.L.; Willis, M.; Barshop, B.A.; Gangoiti, J.
    Positive newborn screen in the biochemically normal infant of a mother with treated holocarboxylase synthetase deficiency (2009), J. Inherit. Metab. Dis., 32, S79-82.
    View publication on PubMed

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
ATP + biotin + apo-[propionyl-CoA carboxylase] Homo sapiens
-
AMP + diphosphate + propionyl-CoA carboxylase
-
?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
ATP + biotin + apo-[propionyl-CoA carboxylase]
-
Homo sapiens AMP + diphosphate + propionyl-CoA carboxylase
-
?

Synonyms

Synonyms Comment Organism
HCS
-
Homo sapiens
Holocarboxylase synthetase
-
Homo sapiens

General Information

General Information Comment Organism
malfunction holocarboxylase synthetase deficiency causes multiple carboxylase deficiency, phenotypes, overview Homo sapiens
metabolism the enzyme is part of the biotin metabolism in which it catalyses the attachment of biotin to apoenzyme carboxylases Homo sapiens