Ph.D. candidate in Chonglin Yang's lab
IGDB, Chinese Academy of Sciences
Host: Johan Auwerx
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Mitochondria are central to cell homeostasis by coordinating many catabolic and anabolic pathways and coupling the TCA cycle with ATP production. However, it is largely unknown how aberrant metabolism affect mitochondria. Here we show the requirement for mitochondrial saccharopine degradation in mitochondrial homeostasis and animal development, which provides mechanistic and therapeutic insights for understanding and treating hyperlysinemia II (saccharopinuria), an aminoacidopathy with severe developmental defects. Besides, we demonstrate that defective degradation of D-2HG induces mitochondrial damage by inhibiting the metabolism of propionate, suggesting that mitochondrial dysfunction contributes to D-2HG related metabolic disorders and cancers.