Saccharopine (L-Saccharopine), a lysine degradation intermediate, is a mitochondrial toxin. Lysine and α-ketoglutarate are converted into Saccharopine by the lysine-ketoglutarate reductase. Saccharopine is then oxidized to α-aminoapidate semialdehyde and glutamate by the saccharopine dehydrogenase. Saccharopine impairs development by disrupting mitochondrial homeostasis[2].In Vitro:Saccharopine accumulation leads to mitochondrial damage and functional loss which is induced by saccharopine dehydrogenase (SDH) mutations of α-aminoadipate semialdehyde synthase (AASS)-1 in C. elegans.In Vivo:Saccharopine accumulation induces mitochondrial damage and progressive postnatal growth retardation in Aass mutant mice.