Flavin adenine dinucleotide binding; dihydrolipoyl dehydrogenase activity; electron transfer activity. It is involved in the biological process described with: tricarboxylic acid cycle; lipoamide metabolic process; oxidation-reduction process; glycine catabolic process; cell redox homeostasis.

Midline uncoordinated, isoform B; Pyruvate dehydrogenase (NAD+) activity; dihydrolipoyllysine-residue acetyltransferase activity. It is involved in the biological process described with: grooming behavior; acetyl-CoA biosynthetic process from pyruvate.

Succinate--CoA ligase [ADP-forming] subunit beta, mitochondrial; ATP-specific succinyl-CoA synthetase functions in the citric acid cycle (TCA), coupling the hydrolysis of succinyl-CoA to the synthesis of ATP and thus represents the only step of substrate-level phosphorylation in the TCA. The beta subunit provides nucleotide specificity of the enzyme and binds the substrate succinate, while the binding sites for coenzyme A and phosphate are found in the alpha subunit.

Isocitrate dehydrogenase (Idh) encodes a cytosolic enzyme that catalyzes the oxidative decarboxylation of isocitrate to 2-oxoglutarate, with the concomitant reduction of NADP[+] to NADPH. It functions, along with Men, Zw, and Pgd, in a small network of NADP reducing enzymes.

Aconitate hydratase, mitochondrial; 4 iron, 4 sulfur cluster binding; aconitate hydratase activity. It is involved in the biological process described with: tricarboxylic acid cycle; multicellular organism reproduction; Belongs to the aconitase/IPM isomerase family.

Dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex; Acetyltransferase activity; lipoic acid binding.

Succinate--CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial; Succinyl-CoA synthetase functions in the citric acid cycle (TCA), coupling the hydrolysis of succinyl-CoA to the synthesis of either ATP or GTP and thus represents the only step of substrate-level phosphorylation in the TCA. The alpha subunit of the enzyme binds the substrates coenzyme A and phosphate, while succinate binding and specificity for either ATP or GTP is provided by different beta subunits.

Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial; Flavoprotein (FP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q). Maintaining electron transport chain function is required to prevent neurodegenerative changes seen in both early- and late-onset disorders.

Citrate (Si)-synthase activity. It is involved in the biological process described with: carbohydrate metabolic process; tricarboxylic acid cycle.