Abstract
Mitochondria are peculiar organelles whose proper function depends on the crosstalk between two genomes, mitochondrial and nuclear. The human mitochondrial genome (mtDNA) encodes only 13 proteins; nevertheless, its proper expression is essential for cellular homeostasis, as mtDNA-encoded proteins are constituents of mitochondrial respiratory complexes. In addition, mtDNA expression results in the production of RNA molecules, which influence cell physiology once released from the mitochondria into the cytoplasm. As a result, dysfunctions of mtDNA expression may lead to pathologies in humans. Here, we review the mechanisms of mitochondrial gene expression with a focus on recent findings in the field. We summarize the complex turnover of mitochondrial transcripts and present an increasing body of evidence indicating new functions of mitochondrial transcripts. We discuss mitochondrial gene regulation in different cellular contexts, focusing on stress conditions. Finally, we highlight the importance of emerging aspects of mitochondrial gene regulation in human health and disease.
Highlights
The Role of Mitochondria within the CellMitochondria are present in the majority of eukaryotic cells, where they play a central role in many processes
An interesting example pertains to mitochondrial transcription rescue factor 1 (MTRES1), which was shown to interact with POLRMT and TFAM and to prevent stress-induced loss of mitochondrial RNA (mtRNA) by acting at the mitochondrial transcription initiation level [81]
Another example refers to mitochondrial ribosomal protein L7/L12 (MRPL12), whose mutations may lead to respiratory chain deficiency that manifest as growth retardation and neurological deterioration [82]
Summary
Mitochondria are present in the majority of eukaryotic cells, where they play a central role in many processes. They are hubs of energy production as sites of oxidative phosphorylation [1]. Cells 2020, 9, 17 dozen proteins that are parts of the oxidative phosphorylation complexes encoded by the mitochondrial genome [18,19]. New findings deciphering the link between mitochondrial gene expression and cellular homeostasis were reported. We review the basis of mitochondrial gene regulation with a focus on recent findings, highlighting new RNA-driven mechanisms by which mitochondria contribute to the regulation of cell biology in human health and disease
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