Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
mitochondrion | - |
Mus musculus | 5739 | - |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
3-methyl-2-oxobutanoate + [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine | Mus musculus | - |
[dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine + CO2 | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Mus musculus | - |
- |
- |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
liver | - |
Mus musculus | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
3-methyl-2-oxobutanoate + [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine | - |
Mus musculus | [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine + CO2 | - |
? |
Synonyms | Comment | Organism |
---|---|---|
BCKDH | - |
Mus musculus |
branched chain alpha-ketoacid dehydrogenase complex | - |
Mus musculus |
Cofactor | Comment | Organism | Structure |
---|---|---|---|
CoA | - |
Mus musculus | |
NAD+ | - |
Mus musculus |
Organism | Comment | Expression |
---|---|---|
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 |
General Information | Comment | Organism |
---|---|---|
malfunction | maple syrup urine disease is an inborn error of metabolism of the branched chain alpha-ketoacid dehydrogenase complex | Mus musculus |