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Literature summary for 6.4.1.4 extracted from

  • Arnold, G.L.; Koeberl, D.D.; Matern, D.; Barshop, B.; Braverman, N.; Burton, B.; Cederbaum, S.; Fiegenbaum, A.; Garganta, C.; Gibson, J.; Goodman, S.I.; Harding, C.; Kahler, S.; Kronn, D.; Longo, N.
    A Delphi-based consensus clinical practice protocol for the diagnosis and management of 3-methylcrotonyl CoA carboxylase deficiency (2008), Mol. Genet. Metab., 93, 363-370.
    View publication on PubMed
Search for more literature for 6.4.1.4

Metals/Ions

Metals/Ions Comment Organism Structure
Mg2+
-
 Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
ATP + 3-methylcrotonoyl-CoA + HCO3- Homo sapiens a step in the metabolism of leucine into 3-methylcrotonyl CoA and related metabolites, overview ADP + phosphate + 3-methylglutaconyl-CoA
-
 ?
additional information Homo sapiens children with 3-MCC deficiency show mental retardation or metabolic disturbances including ketoacidosis, hypoglycemia, or Reye syndrome, but 3-MCC deficiency can also occur in asymptomatic individuals or as non-specific fasting intolerance with normal neurological outcome, overview. The deficiency is diagnozed by 3-methylcrotonylglycine content in the urine ?
-
 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
ATP + 3-methylcrotonoyl-CoA + HCO3-
-
 Homo sapiens ADP + phosphate + 3-methylglutaconyl-CoA
-
 ?
ATP + 3-methylcrotonoyl-CoA + HCO3- a step in the metabolism of leucine into 3-methylcrotonyl CoA and related metabolites, overview Homo sapiens ADP + phosphate + 3-methylglutaconyl-CoA
-
 ?
additional information children with 3-MCC deficiency show mental retardation or metabolic disturbances including ketoacidosis, hypoglycemia, or Reye syndrome, but 3-MCC deficiency can also occur in asymptomatic individuals or as non-specific fasting intolerance with normal neurological outcome, overview. The deficiency is diagnozed by 3-methylcrotonylglycine content in the urine Homo sapiens ?
-
 ?

Synonyms

Synonyms Comment Organism
3-MCC
-
 Homo sapiens
3-methylcrotonyl CoA carboxylase
-
 Homo sapiens

Cofactor

Cofactor Comment Organism Structure
ATP
-
 Homo sapiens