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^[1][2][3].

Saccharopine accumulation leads to mitochondrial damage and functional loss which is induced by saccharopine dehydrogenase (SDH) mutations of α-aminoadipate semialdehyde synthase (AASS)-1 inC. elegans^[1].

Saccharopine accumulation induces mitochondrial damage and progressive postnatal growth retardation inAassmutant mice^[1].

276.29

C₁₁H₂₀N₂O₆

997-68-2

L-酵母氨酸；酵母氨酸

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