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