Human mitochondria synthesize 13 subunits of the oxidative respiratory chain complexes through an independent translation system. Impairment of mitochondrial translation affects cellular aerobic respiration and has been linked to various diseases, including heart disease and neurodegenerative disorders. This study discovered the mitochondrial ribosome-associated quality control pathway (mtRQC) and identified disease-related repair factors involved in this process, such as mtRF-R and MTRES1, which are associated with conditions like optic atrophy and peripheral neuropathy. It provides the first biochemical evidence for the existence of this pathway. Additionally, the study observed the Oxa1L-mediated translocation of mitochondrially synthesized membrane proteins, expanding our understanding of the molecular mechanisms underlying membrane-anchored protein synthesis within mitochondria (Science, 2020).
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