EC Number | Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|---|
1.2.4.4 | mitochondrion | - |
Mus musculus | 5739 | - |
EC Number | Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|---|
1.2.4.4 | 3-methyl-2-oxobutanoate + [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine | Mus musculus | - |
[dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine + CO2 | - |
? |
EC Number | Organism | UniProt | Comment | Textmining |
---|---|---|---|---|
1.2.4.4 | Mus musculus | - |
- |
- |
EC Number | Source Tissue | Comment | Organism | Textmining |
---|---|---|---|---|
1.2.4.4 | liver | - |
Mus musculus | - |
EC Number | Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|---|
1.2.4.4 | 3-methyl-2-oxobutanoate + [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine | - |
Mus musculus | [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine + CO2 | - |
? |
EC Number | Synonyms | Comment | Organism |
---|---|---|---|
1.2.4.4 | BCKDH | - |
Mus musculus |
1.2.4.4 | branched chain alpha-ketoacid dehydrogenase complex | - |
Mus musculus |
EC Number | Cofactor | Comment | Organism | Structure |
---|---|---|---|---|
1.2.4.4 | CoA | - |
Mus musculus | |
1.2.4.4 | NAD+ | - |
Mus musculus |
EC Number | Organism | Comment | Expression |
---|---|---|---|
1.2.4.4 | Mus musculus | donor hepatocytes expressing wild type BCKDH engrafted into intermediate maple syrup urine disease recipient liver, increase liver BCKDH activity. Hepatocyte transplantation increases liver BCKDH activity 14.36% of control activity | up |
EC Number | General Information | Comment | Organism |
---|---|---|---|
1.2.4.4 | malfunction | maple syrup urine disease is an inborn error of metabolism of the branched chain alpha-ketoacid dehydrogenase complex | Mus musculus |