Organization and expression of the mammalian mitochondrial genome

O Rackham, A Filipovska - Nature Reviews Genetics, 2022 - nature.com
The mitochondrial genome encodes core subunits of the respiratory chain that drives
oxidative phosphorylation and is, therefore, essential for energy conversion. Advances in …

tRNA dysregulation and disease

EA Orellana, E Siegal, RI Gregory - Nature Reviews Genetics, 2022 - nature.com
Abstract tRNAs are key adaptor molecules that decipher the genetic code during translation
of mRNAs in protein synthesis. In contrast to the traditional view of tRNAs as ubiquitously …

Structural basis for human mitochondrial tRNA maturation

V Meynier, SW Hardwick, M Catala, JJ Roske… - Nature …, 2024 - nature.com
The human mitochondrial genome is transcribed into two RNAs, containing mRNAs, rRNAs
and tRNAs, all dedicated to produce essential proteins of the respiratory chain. The precise …

Advances in the Structural and Functional Understanding of m1A RNA Modification

J Smoczynski, MJ Yared, V Meynier… - Accounts of Chemical …, 2024 - ACS Publications
Conspectus RNA modification is a co-or post-transcriptional process by which specific
nucleotides are chemically altered by enzymes after their initial incorporation into the RNA …

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 …

Starting the engine of the powerhouse: mitochondrial transcription and beyond

M Miranda, NA Bonekamp, I Kühl - Biological Chemistry, 2022 - degruyter.com
Mitochondria are central hubs for cellular metabolism, coordinating a variety of metabolic
reactions crucial for human health. Mitochondria provide most of the cellular energy via their …

Deficiency of the mitochondrial ribosomal subunit, MRPL50, causes autosomal recessive syndromic premature ovarian insufficiency

S Bakhshalizadeh, DH Hock, NA Siddall, BL Kline… - Human Genetics, 2023 - Springer
Premature ovarian insufficiency (POI) is a common cause of infertility in women,
characterised by amenorrhea and elevated FSH under the age of 40 years. In some cases …

Cytosolic N6AMT1-dependent translation supports mitochondrial RNA processing

MM Foged, E Recazens, S Chollet, M Lisci… - Proceedings of the …, 2024 - pnas.org
Mitochondrial biogenesis relies on both the nuclear and mitochondrial genomes, and
imbalance in their expression can lead to inborn errors of metabolism, inflammation, and …

Multiple structural flavors of RNase P in precursor tRNA processing

S Sridhara - Wiley Interdisciplinary Reviews: RNA, 2024 - Wiley Online Library
The precursor transfer RNAs (pre‐tRNAs) require extensive processing to generate mature
tRNAs possessing proper fold, structural stability, and functionality required to sustain …

Mitochondrial dysfunction in cardiac diseases and therapeutic strategies

Y Huang, B Zhou - Biomedicines, 2023 - mdpi.com
Mitochondria are the main site of intracellular synthesis of ATP, which provides energy for
various physiological activities of the cell. Cardiomyocytes have a high density of …