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Literature summary extracted from
- My favorite pyruvate carboxylase (2010), IUBMB Life, 62, 535-538.
Activating Compound
| EC Number | Activating Compound | Comment | Organism | Structure |
|---|---|---|---|---|
| 6.4.1.1 | acetyl-CoA | - |
Homo sapiens |  |
| 6.4.1.1 | acetyl-CoA | - |
Rattus norvegicus | |
Metals/Ions
| EC Number | Metals/Ions | Comment | Organism | Structure |
|---|---|---|---|---|
| 6.4.1.1 | Mg2+ | required for activity | Homo sapiens | |
| 6.4.1.1 | Mg2+ | required for activity | Rattus norvegicus | |
Organism
| EC Number | Organism | UniProt | Comment | Textmining |
|---|---|---|---|---|
| 6.4.1.1 | Homo sapiens | - |
- |
- |
| 6.4.1.1 | Rattus norvegicus | - |
- |
- |
Substrates and Products (Substrate)
| EC Number | Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|---|
| 6.4.1.1 | ATP + pyruvate + HCO3- + H+ | - |
Homo sapiens | ADP + oxaloacetate + phosphate | - |
r | |
| 6.4.1.1 | ATP + pyruvate + HCO3- + H+ | - |
Rattus norvegicus | ADP + oxaloacetate + phosphate | - |
r | |
Subunits
| EC Number | Subunits | Comment | Organism |
|---|---|---|---|
| 6.4.1.1 | homotetramer | - |
Homo sapiens |
| 6.4.1.1 | homotetramer | - |
Rattus norvegicus |
Cofactor
| EC Number | Cofactor | Comment | Organism | Structure |
|---|---|---|---|---|
| 6.4.1.1 | ATP | - |
Homo sapiens | |
| 6.4.1.1 | ATP | - |
Rattus norvegicus | |
| 6.4.1.1 | biotin | - |
Homo sapiens | |
| 6.4.1.1 | biotin | - |
Rattus norvegicus | |
Expression
| EC Number | Organism | Comment | Expression |
|---|---|---|---|
| 6.4.1.1 | Rattus norvegicus | expression of pyruvate carboxylase is be increased 2-5fold at the onset of obesity in Zucker fatty rats | up |
General Information
| EC Number | General Information | Comment | Organism |
|---|---|---|---|
| 6.4.1.1 | malfunction | pyruvate decarboxylase deficiency type A is characterised by hypoglycemia accompanied by mild to moderate lactic acidemia and sometimes elevated ketone body levels. Type B, having no detectable pyruvate carboxylase protein in any tissues, is the most severe form which leads to death generally within three months from lactic academia accompanied by hyperammonemia, citrullinemia and hyperlysinemia. Type C has a benign phenotype associated with episodes of lactic acidemia and no psychomotor disorders | Homo sapiens |
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Release 2023.1 (January 2023)
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