[HTML][HTML] Human mitochondrial RNA processing and modifications: overview

…, A Jabczynska, RJ Szczesny - International Journal of …, 2021 - mdpi.com
Mitochondria, often referred to as the powerhouses of cells, are vital organelles that are present
in almost all eukaryotic organisms, including humans. They are the key energy suppliers …

Mitochondrial double-stranded RNA triggers antiviral signalling in humans

…, J Rehwinkel, A Dziembowski, RJ Szczesny… - Nature, 2018 - nature.com
Mitochondria are descendants of endosymbiotic bacteria and retain essential prokaryotic
features such as a compact circular genome. Consequently, in mammals, mitochondrial DNA is …

[HTML][HTML] Mitochondrial gene expression and beyond—novel aspects of cellular physiology

AV Kotrys, RJ Szczesny - Cells, 2019 - mdpi.com
Mitochondria are peculiar organelles whose proper function depends on the crosstalk
between two genomes, mitochondrial and nuclear. The human mitochondrial genome (mtDNA) …

The human core exosome interacts with differentially localized processive RNases: hDIS3 and hDIS3L

…, A Chlebowski, KM Larsen, RJ Szczesny… - The EMBO …, 2010 - embopress.org
The eukaryotic RNA exosome is a ribonucleolytic complex involved in RNA processing and
turnover. It consists of a nine‐subunit catalytically inert core that serves a structural function …

Involvement of human ELAC2 gene product in 3'end processing of mitochondrial tRNAs

LK Brzezniak, M Bijata, RJ Szczesny, PP Stepien - RNA biology, 2011 - Taylor & Francis
Accurate tRNA processing is crucial for human mitochondrial genome expression, but the
mechanisms of mt-tRNA cleavage and the key enzymes involved in this process are poorly …

Human mitochondrial RNA decay mediated by PNPase–hSuv3 complex takes place in distinct foci

…, MS Hejnowicz, PP Stepien, RJ Szczesny - Nucleic acids …, 2013 - academic.oup.com
RNA decay is usually mediated by protein complexes and can occur in specific foci such as
P-bodies in the cytoplasm of eukaryotes. In human mitochondria nothing is known about the …

Human mitochondrial RNA turnover caught in flagranti: involvement of hSuv3p helicase in RNA surveillance

RJ Szczesny, LS Borowski, LK Brzezniak… - Nucleic acids …, 2010 - academic.oup.com
The mechanism of human mitochondrial RNA turnover and surveillance is still a matter of
debate. We have obtained a cellular model for studying the role of hSuv3p helicase in human …

RNA degradation in yeast and human mitochondria

RJ Szczesny, LS Borowski, M Malecki… - … et Biophysica Acta (BBA …, 2012 - Elsevier
Expression of mitochondrially encoded genes must be finely tuned according to the cell's
requirements. Since yeast and human mitochondria have limited possibilities to regulate gene …

Linear mtDNA fragments and unusual mtDNA rearrangements associated with pathological deficiency of MGME1 exonuclease

…, V Peeva, S Schöler, RJ Szczesny… - Human molecular …, 2014 - academic.oup.com
MGME1, also known as Ddk1 or C20orf72, is a mitochondrial exonuclease found to be
involved in the processing of mitochondrial DNA (mtDNA) during replication. Here, we present …

Multiple myeloma-associated hDIS3 mutations cause perturbations in cellular RNA metabolism and suggest hDIS3 PIN domain as a potential drug target

…, I Kucinski, K Stodus, RJ Szczesny… - Nucleic acids …, 2013 - academic.oup.com
hDIS3 is a mainly nuclear, catalytic subunit of the human exosome complex, containing
exonucleolytic (RNB) and endonucleolytic (PIN) active domains. Mutations in hDIS3 have been …