Abstract
Mitochondria are cytosolic organelles that have many essential roles including ATP production via oxidative phosphorylation, apoptosis, iron‐sulfur cluster biogenesis, heme and steroid synthesis, calcium homeostasis, and regulation of cellular redox state. One of the unique features of these organelles is the presence of an extrachromosomal mitochondrial genome (mtDNA), together with all the machinery required to replicate and transcribe mtDNA. The accurate maintenance of mitochondrial gene expression is essential for correct organellar metabolism, and is in part dependent on the levels of mtDNA and mtRNA, which are regulated by balancing synthesis against degradation. It is clear that although a number of mitochondrial nucleases have been identified, not all those responsible for the degradation of DNA or RNA have been characterized. Recent investigations, however, have revealed the contribution that mutations in the genes coding for these enzymes has made to causing pathogenic mitochondrial diseases.
Highlights
Mitochondria are cytosolic organelles that have many essential roles including ATP production via oxidative phosphorylation, apoptosis, iron-sulfur cluster biogenesis, heme and steroid synthesis, calcium homeostasis, and regulation of cellular redox state
The mitochondrial RNases that have been identified are, predictably, involved in mtRNA processing and degradation; a few may play a role in mitochondrial genome (mtDNA) replication but their exact function in this process is controversial
This review summarizes the recent advances in characterizing mitochondrial nucleases (Fig. 1), and the approach of engineering nucleases to be mitochondrially targeted as a tool to manipulate human mtDNA, with the long-term goal of treating human mitochondrial dysfunction
Summary
The Wellcome Trust Centre for Mitochondrial Research, The Medical School, Newcastle University, UK. Keywords cleavage; degradation; human; mtDNA; mitochondria; mtRNA; nucleases; processing. Mitochondria are cytosolic organelles that have many essential roles including ATP production via oxidative phosphorylation, apoptosis, ironsulfur cluster biogenesis, heme and steroid synthesis, calcium homeostasis, and regulation of cellular redox state. The accurate maintenance of mitochondrial gene expression is essential for correct organellar metabolism, and is in part dependent on the levels of mtDNA and mtRNA, which are regulated by balancing synthesis against degradation. Recent investigations have revealed the contribution that mutations in the genes coding for these enzymes has made to causing pathogenic mitochondrial diseases
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