Interplay between substrate recognition, 5′ end tRNA processing and methylation activity of human mitochondrial RNase P

A Karasik, CA Fierke, M Koutmos - Rna, 2019 - rnajournal.cshlp.org
Human mitochondrial ribonuclease P (mtRNase P) is an essential three-protein complex
that catalyzes the 5′ end maturation of mitochondrial precursor tRNAs (pre-tRNAs) …

Structural basis of RNA processing by human mitochondrial RNase P

A Bhatta, C Dienemann, P Cramer… - Nature Structural & …, 2021 - nature.com
Human mitochondrial transcripts contain messenger and ribosomal RNAs flanked by
transfer RNAs (tRNAs), which are excised by mitochondrial RNase (mtRNase) P and Z to …

Kinetic mechanism of human mitochondrial RNase P

X Liu, N Wu, A Shanmuganathan, BP Klemm… - bioRxiv, 2019 - biorxiv.org
ABSTRACT A first step in processing mitochondrial precursor tRNA (pre-tRNA) is cleavage
of the 5'leader catalyzed by ribonuclease P (RNase P). Human mitochondrial RNase P …

Disease-associated mutations in mitochondrial precursor tRNAs affect binding, m1R9 methylation, and tRNA processing by mtRNase P

A Karasik, CA Wilhelm, CA Fierke, M Koutmos - RNA, 2021 - rnajournal.cshlp.org
Mitochondrial diseases linked to mutations in mitochondrial (mt) tRNA sequences are
common. However, the contributions of these tRNA mutations to the development of …

Cleavage kinetics of human mitochondrial RNase P and contribution of its non-nuclease subunits

E Vilardo, U Toth, E Hazisllari… - Nucleic Acids …, 2023 - academic.oup.com
RNase P is the endonuclease responsible for the 5′ processing of precursor tRNAs (pre-
tRNAs). Unlike the single-subunit protein-only RNase P (PRORP) found in plants or protists …

A subcomplex of human mitochondrial RNase P is a bifunctional methyltransferase—extensive moonlighting in mitochondrial tRNA biogenesis

E Vilardo, C Nachbagauer, A Buzet… - Nucleic acids …, 2012 - academic.oup.com
Transfer RNAs (tRNAs) reach their mature functional form through several steps of
processing and modification. Some nucleotide modifications affect the proper folding of …

Structure of the nuclease subunit of human mitochondrial RNase P

L Reinhard, S Sridhara, BM Hällberg - Nucleic Acids Research, 2015 - academic.oup.com
Mitochondrial RNA polymerase produces long polycistronic precursors that contain the
mRNAs, rRNAs and tRNAs needed for mitochondrial translation. Mitochondrial RNase P (mt …

Structural insight into the human mitochondrial tRNA purine N1-methyltransferase and ribonuclease P complexes

S Oerum, M Roovers, RP Rambo, J Kopec… - Journal of Biological …, 2018 - ASBMB
Mitochondrial tRNAs are transcribed as long polycistronic transcripts of precursor tRNAs and
undergo posttranscriptional modifications such as endonucleolytic processing and …

The MRPP1/MRPP2 complex is a tRNA-maturation platform in human mitochondria

L Reinhard, S Sridhara, BM Hällberg - Nucleic acids research, 2017 - academic.oup.com
Mitochondrial polycistronic transcripts are extensively processed to give rise to functional
mRNAs, rRNAs and tRNAs; starting with the release of tRNA elements through 5 …

Loss of individual mitochondrial ribonuclease P complex proteins differentially affects mitochondrial tRNA processing in vivo

M Saoji, A Sen, RT Cox - International Journal of Molecular Sciences, 2021 - mdpi.com
Over a thousand nucleus-encoded mitochondrial proteins are imported from the cytoplasm;
however, mitochondrial (mt) DNA encodes for a small number of critical proteins and the …