Metals/Ions | Comment | Organism | Structure |
---|---|---|---|
Mg2+ | - |
Homo sapiens |
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 | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
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 | Comment | Organism |
---|---|---|
3-MCC | - |
Homo sapiens |
3-methylcrotonyl CoA carboxylase | - |
Homo sapiens |
Cofactor | Comment | Organism | Structure |
---|---|---|---|
ATP | - |
Homo sapiens |