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Link to original content: http://www.ncbi.nlm.nih.gov/pubmed/31064115
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Review
. 2019 May 6;20(9):2221.
doi: 10.3390/ijms20092221.

Transcription, Processing, and Decay of Mitochondrial RNA in Health and Disease

Arianna Barchiesi  1   2 , Carlo Vascotto  3   4
Affiliations
Review

Transcription, Processing, and Decay of Mitochondrial RNA in Health and Disease

Arianna Barchiesi et al. Int J Mol Sci. .

Abstract

Although the large majority of mitochondrial proteins are nuclear encoded, for their correct functioning mitochondria require the expression of 13 proteins, two rRNA, and 22 tRNA codified by mitochondrial DNA (mtDNA). Once transcribed, mitochondrial RNA (mtRNA) is processed, mito-ribosomes are assembled, and mtDNA-encoded proteins belonging to the respiratory chain are synthesized. These processes require the coordinated spatio-temporal action of several enzymes, and many different factors are involved in the regulation and control of protein synthesis and in the stability and turnover of mitochondrial RNA. In this review, we describe the essential steps of mitochondrial RNA synthesis, maturation, and degradation, the factors controlling these processes, and how the alteration of these processes is associated with human pathologies.

Keywords: RNA degradation; RNA processing; RNA transcription; mitochondria; mitochondrial diseases.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Schematic representation of mitochondrial D-loop region. Heavy strand promoters 1 and 2 (HSP1 and HSP1), light strand promoter 1 (LSP1), conserved sequence blocks 1, 2, and 3 (CSB I, II, and III), and termination-associated sequences (TAS).
Figure 2
Figure 2
Schematic view of mitochondrial DNA transcription, RNA processing, and degradation. Mitochondrial DNA transcription takes place between nucleoids and mitochondrial RNA granules (MRGs). TFAM is the principal protein needed for the initiation of transcription as long as POLMRT, TFBM1, and TFBM2 (1). After transcription of the polycistronic molecules, RNA is immediately processed by RNASE P and Z to release tRNAs, following the tRNA punctuation model. Once tRNAs, mRNAs, and rRNAs are released, the translation process can start (2). mRNA degradation takes place in the D-foci, close to the MRGs. PNPase and hSuv3 are components of the degradosome. REXO2 isalso present in this compartment and degrades small RNA oligonucleotides (3). Black arrows: transitions; Red arrow: inhibition.
See this image and copyright information in PMC

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