Dihydrolipoyl dehydrogenase (DLD), the E3 component of the b...

created [InstanceEdit:9859255] Stephan, Ralf, 2024-01-17
dbId 9859254
displayName Dihydrolipoyl dehydrogenase (DLD), the E3 component of the b...
modified [InstanceEdit:9907759] Stephan, Ralf, 2024-04-17
schemaClass Summation
text Dihydrolipoyl dehydrogenase (DLD), the E3 component of the branched-chain alpha-keto acid dehydrogenase complex (BCKDH), forms a homodimer that catalyzes the dehydrogenation of dihydrolipoyllysine residues by producing NADH from NAD+, with the help of the FAD cofactor (Brautigam et al., 2005). This reaction, in a side reaction not annotated here, is the main source of reactive oxygen species (ROS) in mitochondria (Ambrus et al., 2015). Deficiency of DLD activity due to mutations is a rare genetic disease characterized by neonatal lactic acidosis due to lack of pyruvate dehydrogenase activity, although symptoms of BCKDH deficiency may also be present (DLDD; MIM:246900; see Wongkittichote et al., 2023; reviewed in Ambrus & Adam-Viz, 2017).
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