Mitochondrial RNase P Complex in Animals: Mitochondrial tRNA Processing and Links to Disease

  • Maithili Saoji
  • Rachel T. CoxEmail author
Part of the Nucleic Acids and Molecular Biology book series (NUCLEIC, volume 34)


Loss of mitochondrial function not only causes specific mitochondrial diseases but also contributes to serious conditions such as neurodegeneration and diabetes. Since mitochondrial DNA is transcribed as a polycistronic message comprised of three forms of RNA (rRNA, mRNA, and tRNA), proper 5′- and 3′-end cleavage is essential. In the nucleus, tRNA 5′-end processing is carried out by the first identified ribozyme, RNase P. In contrast, mitochondrial tRNAs are processed by a three-protein complex, mitochondrial RNase P, which does not have an RNA component. An accessory subcomplex made of the m1A9 methyltransferase MRPP1 and the dehydrogenase MRPP2 binds to the metallonuclease MRPP3 that cleaves the RNA phosphodiester backbone. Each protein has been shown to be essential in model organisms, and loss of each gives rise to human multisystemic diseases with many characteristics of mitochondrial disease. In this review, we discuss what is known about the mitochondrial RNase P complex, the molecular mechanism of 5′-end mitochondrial tRNA processing, and how loss of this activity causes human disease.



This work was supported by the National Institutes of Health/Department of Defense [CHIRP HU0001–14–2-0041 to M.S. and R.T.C.].


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© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Biochemistry and Molecular BiologyThe Collaborative Health Initiative Research Program (CHIRP), Uniformed Services UniversityBethesdaUSA

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