DLD dimer dehydrogenates dihydrolipoyl
NAD+ + LipoH2-K105-DBT → H+ + NADH + Lipo-K105-DBT
- Reactome
- R-HSA-9859172
- Compartment
- mitochondrial matrix
- Rhea
- RHEA:15046
Inputs
- NAD+CHEBI:57540, C21H26N7O14P2, mitochondrial matrix
- LipoH2-K105-DBTR-HSA-9859167, mitochondrial matrix
Outputs
- H+CHEBI:15378, H, mitochondrial matrix
- NADHCHEBI:57945, C21H27N7O14P2, mitochondrial matrix
- Lipo-K105-DBTR-HSA-70017, mitochondrial matrix
Catalysed by
- dihydrolipoyl dehydrogenase (NADH) activity of BCKDH [mitochondrial matrix]EC 1.8.1.4
Literature
- Crystal structure of human dihydrolipoamide dehydrogenase: NAD+/NADH binding and the structural basis of disease-causing mutations J Mol Biol, 2005
- Human dihydrolipoamide dehydrogenase (E3) deficiency: Novel insights into the structural basis and molecular pathomechanism Neurochem Int, 2018
- Formation of reactive oxygen species by human and bacterial pyruvate and 2-oxoglutarate dehydrogenase multienzyme complexes reconstituted from recombinant components Free Radic Biol Med, 2015
- Biochemical characterization of patients with dihydrolipoamide dehydrogenase deficiency JIMD Rep, 2023