Figure | Mitochondrial RNA metabolism. Image based on 
The human mitochondrial genome (mtDNA) encodes several structural components of the oxidative phosphorylation system (OxPhos) and RNA components for intramitochondrial protein synthesis. Therefore, mitochondria have evolved unique and highly specialised mechanisms to express genes contained within mtDNA .
The mitochondrial rRNAs (blue), mRNAs (red) and tRNAs (green) are transcribed as polycistronic units. Following the endonucleolytic processing, individual mRNA, rRNA and tRNA transcripts undergo post-transcriptional modifications. Several nucleotides of rRNAs are modified to facilitate mitoribosome biogenesis and function, a poly(A) tail is added to mRNAs and tRNA and rRNA undergo extensive post-transcriptional nucleotide modification, in addition to a CCA trinucleotide synthesis at the 3’ end, before being aminoacylated with a cognate amino acid. Decay and surveillance pathways have also been described for mammalian mtRNA  .
Establishing how defects in these processes contribute to human disease constitutes a major challenge. Recent years have brought many important insights into the regulation of mtDNA maintenance and expression. A number of novel factors have been identified, either by basic research approaches, or through the study of patients with respiratory chain disorders. The characterisation of these factors has furthered a basic understanding of their function in processes of mitochondrial DNA (mtDNA) replication, mtRNA metabolism and translation, and has also shed new light upon these processes. Nonetheless, many fundamental questions, frequently related to the pathology of mitochondrial diseases, remain unanswered and new questions have arisen.
Currently, we try to understand the regulation of mammalian mitochondrial gene expression. We focus mainly on studying fundamental processes of:
• Endonuclolytic processing of precursor mitochondrial RNA
• Polyadenylation of mitochondrial RNA  
• Post-transcriptional modification (epitranscriptomics) of mitochondrial RNA   
• Mitoribosome biogenesis  
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