Limna - Lausanne Integrative Metabolism Nutrition Alliance

SEMINAR

Wednesday 13th March 2019
11h00 - Room AI 1142 (*)- EPFL Lausanne

Regulation of mitochondrial homeostasis by metabolism in mitochondria


Junxiang ZHOU

Ph.D. candidate in Chonglin Yang's lab

IGDB, Chinese Academy of Sciences

Host: Johan Auwerx

(*) IMPORTANT NOTICE: All external participants have to pass through SV Reception/Welcome Desk to be able to access to AI 1153. 

Abstract

 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.