Fig. 7

GAA inhibits cell proliferation through LRPPRC regulated OXPHOS. LRPPRC interacts with mtRNA, and this interaction increases mtRNA’s stability, thereby promoting the synthesis of oxidative phosphorylation complex subunits during cancer cell proliferation. Competitive binding of GAA and mtRNA to LRPPRC destabilized both mtRNA and LRPPRC simultaneously. This inhibition suppressed the synthesis of oxidative phosphorylation complex subunits, resulting in OXPHOS defects, growth inhibition, and stem cell clearance