Saccharopine hydrochloride (L-Saccharopine hydrochloride), an intermediate in lysine degradation and a mitochondrial toxin, is produced through the conversion of lysine and alpha-ketoglutarate by lysine-ketoglutarate reductase. This compound is subsequently oxidized to alpha-aminoapidate semialdehyde and glutamate by saccharopine dehydrogenase, impairing development by disrupting mitochondrial homeostasis (1) (2) (3).