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Literature summary for 1.8.1.4 extracted from
- Molecular dynamics study of the structural basis of dysfunction and the modulation of reactive oxygen species generation by pathogenic mutants of human dihydrolipoamide dehydrogenase (2013), Arch. Biochem. Biophys., 538, 145-155 .
Natural Substrates/ Products (Substrates)
| Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| dihydrolipoamide + NAD+ | Homo sapiens | - |
lipoamide + NADH + H+ | - |
r |  |
Organism
| Organism | UniProt | Comment | Textmining |
|---|---|---|---|
| Homo sapiens | - |
- |
- |
Substrates and Products (Substrate)
| Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| dihydrolipoamide + NAD+ | - |
Homo sapiens | lipoamide + NADH + H+ | - |
r | |
Subunits
| Subunits | Comment | Organism |
|---|---|---|
| homodimer | - |
Homo sapiens |
Synonyms
| Synonyms | Comment | Organism |
|---|---|---|
| dihydrolipoamide dehydrogenase | - |
Homo sapiens |
| DLD | - |
Homo sapiens |
| E3 | - |
Homo sapiens |
| E3 component | - |
Homo sapiens |
| LADH | - |
Homo sapiens |
Cofactor
| Cofactor | Comment | Organism | Structure |
|---|---|---|---|
| flavin | flavoprotein | Homo sapiens | |
General Information
| General Information | Comment | Organism |
|---|---|---|
| malfunction | pathogenic mutations of LADH cause severe metabolic disturbances, called E3 deficiency that often involve cardiological and neurological symptoms and premature death. Some of the known pathogenic mutations augment the reactive oxygen species (ROS) generation capacity of LADH, which may contribute to the clinical presentations. Structural changes are likely to turn the physiological LADH conformation to its ROS-generating conformation | Homo sapiens |
| additional information | molecular dynamics simulation the conformation of enzyme LADH that is proposed to be compatible with the reactive oxygen species (ROS) generation | Homo sapiens |
| physiological function | dihydrolipoamide dehydrogenase is a component in the pyruvate-, 2-oxooglutarate- and branched-chain oxoacid dehydrogenase complexes and in the glycine cleavage system | Homo sapiens |
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