RNase H1 Regulates Mitochondrial Transcription and Translation via the Degradation of 7S RNA

Frontiers in Genetics

卷:10

Katarzyna Tońska¹  Joanna Rusecka¹  Massimo Zeviani²  Aurelio Reyes² 

[1] Institute of Genetics and Biotechnology, Faculty of Biology, University of Warsaw, Warsaw, Poland;

[2] MRC Mitochondrial Biology Unit, University of Cambridge, Cambridge, United Kingdom;

关键词: mitochondria;    mtDNA;    mitochondrial disease;    RNase H1;    transcription;    translation;   

DOI  :  10.3389/fgene.2019.01393

来源: DOAJ

【 摘 要 】

RNase H1 is able to recognize DNA/RNA heteroduplexes and to degrade their RNA component. As a consequence, it has been implicated in different aspects of mtDNA replication such as primer formation, primer removal, and replication termination, and significant differences have been reported between control and mutant RNASEH1 skin fibroblasts from patients. However, neither mtDNA depletion nor the presence of deletions have been described in skin fibroblasts while still presenting signs of mitochondrial dysfunction (lower mitochondrial membrane potential, reduced oxygen consumption, slow growth in galactose). Here, we show that RNase H1 has an effect on mtDNA transcripts, most likely through the regulation of 7S RNA and other R-loops. The observed effect on both mitochondrial mRNAs and 16S rRNA results in decreased mitochondrial translation and subsequently mitochondrial dysfunction in cells carrying mutations in RNASEH1.

【 授权许可】

Unknown