Mitochondrial RNase P (mtRNase P) cleaves the 5' ends of pre-tRNAs and ELAC2 (RNase Z) cleaves the 3' ends of pre-tRNAs in the L strand transcript

Stable Identifier
Reaction [omitted]
Homo sapiens
Locations in the PathwayBrowser
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RNase P, ELAC2, and additional unknown nucleases cleave L strand transcripts to release the tRNAs and an mRNA contained in the long polycistronic transcripts.
Mitochondrial RNase P, comprising 3 protein subunits and no RNA moiety (Holzmann et al. 2008), endonucleolytically cleaves polycistronic mitochondrial transcripts at the 5' ends of the tRNA sequences (Sanchez et al. 2011, Howard et al. 2012, Vilardo et al. 2012, Li et al. 2015, Reinhard et al. 2015, Vilardo and Rossmanith 2015). A subcomplex of RNase P also functions as a tRNA methyltransferase and the SDR5C1 subunit is an amino acid and fatty acid dehydrogenase. Mutations in the SDR5C1 subunit of RNase P cause HSD10 disease, which is characterized by progressive neurodegeneration and cardiomyopathy (Vilardo and Rossmanith 2015)
ELAC2 cleaves polycistronic mitochondrial transcripts at the 3' ends of the tRNA sequences (Brzezniak et al. 2011, Sanchez et al. 2011). Different isoforms of ELAC2 are present in the nucleus and mitochondria (Rossmanith 2011). Mutations in ELAC2 cause cardiac hypertrophy (Haack et al. 2013).
Unknown nucleases also cleave the L strand transcript at a site 3' to MT-ND6 (reviewed in Van Haute et al. 2015).
Literature References
PubMed ID Title Journal Year
23042678 A subcomplex of human mitochondrial RNase P is a bifunctional methyltransferase--extensive moonlighting in mitochondrial tRNA biogenesis

Taschner, A, Vilardo, E, Rossmanith, W, Holzmann, J, Buzet, A, Nachbagauer, C

Nucleic Acids Res. 2012
25925575 Molecular insights into HSD10 disease: impact of SDR5C1 mutations on the human mitochondrial RNase P complex

Vilardo, E, Rossmanith, W

Nucleic Acids Res. 2015
21593607 Involvement of human ELAC2 gene product in 3' end processing of mitochondrial tRNAs

Bijata, M, Brzezniak, LK, Stepien, PP, Szczesny, RJ

RNA Biol 2011
21857155 RNA processing in human mitochondria

Mattick, JS, Mercer, TR, Filipovska, A, Sanchez, MI, Shearwood, AM, Richman, TR, Davies, SM, Rackham, O, Nygård, KK

Cell Cycle 2011
21559454 Localization of human RNase Z isoforms: dual nuclear/mitochondrial targeting of the ELAC2 gene product by alternative translation initiation

Rossmanith, W

PLoS ONE 2011
26016801 Mitochondrial transcript maturation and its disorders

Powell, CA, Minczuk, M, Van Haute, L, Nicholls, TJ, Pearce, SF, D'Souza, AR

J. Inherit. Metab. Dis. 2015
23849775 ELAC2 mutations cause a mitochondrial RNA processing defect associated with hypertrophic cardiomyopathy

Minczuk, M, Mayr, JA, Taylor, RW, Meitinger, T, Rorbach, J, Kopajtich, R, Husain, RA, Mundy, H, Walther, A, Wieland, T, Ferrero, I, Zimmermann, FA, Freisinger, P, Haack, TB, Nicholls, TJ, Baruffini, E, Prokisch, H, Danhauser, K, Strom, TM, Schum, J

Am. J. Hum. Genet. 2013
22336717 Pathogenesis-related mutations in the T-loops of human mitochondrial tRNAs affect 3' end processing and tRNA structure

Serjanov, D, Levinger, L

RNA Biol 2012
18984158 RNase P without RNA: identification and functional reconstitution of the human mitochondrial tRNA processing enzyme

Bennett, KL, Löffler, E, Rossmanith, W, Gerner, C, Holzmann, J, Frank, P

Cell 2008
22991464 Mitochondrial ribonuclease P structure provides insight into the evolution of catalytic strategies for precursor-tRNA 5' processing

Koutmos, M, Lim, WH, Fierke, CA, Howard, MJ

Proc. Natl. Acad. Sci. U.S.A. 2012
25928769 Auto-inhibitory Mechanism of the Human Mitochondrial RNase P Protein Complex

Zhou, W, Li, F, Liu, X, Yang, X, Shen, Y

Sci Rep 2015
25953853 Structure of the nuclease subunit of human mitochondrial RNase P

Reinhard, L, Sridhara, S, Hallberg, BM

Nucleic Acids Res. 2015
Event Information
Catalyst Activity

tRNA-specific ribonuclease activity of ELAC2 [mitochondrial matrix]

This event is regulated
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