线粒体DNA
基因组不稳定性
生物
人类线粒体遗传学
线粒体
遗传学
抄写(语言学)
细胞生物学
基因组
核糖核酸
人类基因组
DNA
DNA损伤
基因
语言学
哲学
作者
Sonia Cristina Pinela da Silva,Lola P. Camino,Andrés Aguilera
标识
DOI:10.1073/pnas.1807258115
摘要
Significance R loops form during transcription when the mRNA hybridizes back to the template DNA forming a stable DNA–RNA hybrid. Stable R loops can block replication and transcription machineries, leading to genome instability and human diseases. We report that the human mitochondrial degradosome (mtEXO), formed by SUV3 and PNPase, plays an important role in R-loop metabolism in the mitochondria apart from preventing dsRNA accumulation, with implications in replication and preventing instability of the mitochondrial genome. Our findings help establish a connection between the RNA processing activities of the mtEXO and mitochondrial genome stability, affecting mitochondria homeostasis, cellular metabolism, and human diseases.
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