Inactivation of the mouse pyruvate dehydrogenase Pdha1 gene is embryonic-lethal [51]. D. rerio mutants with visual function defects – no optokinetic response a (noa) – are deficient in dihydrolipoamide-Sacetyltransferase (Dlat), the PDH E2 subunit [16]: • a phenotype similar to pyruvate dehydrogenase complex deficiency syndrome in humans (neurological dysfunction, lactic acidosis, growth retardation, early death). Here, PDP1 is linked to lactic acidosis.