Import of non-coding RNAs into human mitochondria: a critical review and emerging approaches

D Jeandard, A Smirnova, I Tarassov, E Barrey… - Cells, 2019 - mdpi.com
Mitochondria harbor their own genetic system, yet critically depend on the import of a
number of nuclear-encoded macromolecules to ensure their expression. In all eukaryotes …

Current perspectives on the clinical implications of oxidative RNA damage in aging research: challenges and opportunities

Z Xu, J Huang, M Gao, G Guo, S Zeng, X Chen… - Geroscience, 2021 - Springer
Ribonucleic acid (RNA) molecules can be easily attacked by reactive oxygen species
(ROS), which are produced during normal cellular metabolism and under various oxidative …

Polyribonucleotide nucleotidyltransferase 1 participates in metabolic-associated fatty liver disease pathogenesis by affecting lipid metabolism and mitochondrial …

C Guan, X Zou, C Yang, W Shi, J Gao, Y Ge, Z Xu… - Molecular …, 2024 - Elsevier
Objective Metabolic-associated fatty liver disease (MAFLD) represents one of the most
prevalent chronic liver conditions worldwide, but its precise pathogenesis remains unclear …

Targeting of CRISPR-Cas12a crRNAs into human mitochondria

N Nikitchina, E Ulashchik, V Shmanai, AM Heckel… - Biochimie, 2024 - Elsevier
Mitochondrial gene editing holds great promise as a therapeutic approach for mitochondrial
diseases caused by mutations in the mitochondrial DNA (mtDNA). Current strategies focus …

PNPT1 mutations may cause Aicardi-Goutières-Syndrome

D Bamborschke, M Kreutzer, A Koy, F Koerber… - Brain and …, 2021 - Elsevier
Abstract Background Aicardi-Goutières syndrome (AGS) is a clinically and genetically
heterogenous autoinflammatory disorder caused by constitutive activation of the type I …

Heterozygous PNPT1 Variants Cause Spinocerebellar Ataxia Type 25

M Barbier, M Bahlo, A Pennisi, M Jacoupy… - Annals of …, 2022 - Wiley Online Library
Objective Dominant spinocerebellar ataxias (SCA) are characterized by genetic
heterogeneity. Some mapped and named loci remain without a causal gene identified. Here …

How RNases shape mitochondrial transcriptomes

J Cartalas, L Coudray, A Gobert - International Journal of Molecular …, 2022 - mdpi.com
Mitochondria are the power houses of eukaryote cells. These endosymbiotic organelles of
prokaryote origin are considered as semi-autonomous since they have retained a genome …

Activity and function in human cells of the evolutionary conserved exonuclease polynucleotide phosphorylase

FA Falchi, R Pizzoccheri, F Briani - International Journal of Molecular …, 2022 - mdpi.com
Polynucleotide phosphorylase (PNPase) is a phosphorolytic RNA exonuclease highly
conserved throughout evolution. Human PNPase (hPNPase) is located in mitochondria and …

Clinical Spectrum and Functional Consequences Associated with Bi-Allelic Pathogenic PNPT1 Variants

R Rius, NJ Van Bergen, AG Compton, LG Riley… - Journal of clinical …, 2019 - mdpi.com
PNPT1 (PNPase—polynucleotide phosphorylase) is involved in multiple RNA processing
functions in the mitochondria. Bi-allelic pathogenic PNPT1 variants cause heterogeneous …

Mitochondrial Sequencing Identifies Long Non-Coding RNA Features that Promote Binding to PNPase

AD Taylor, QA Hathaway, A Kunovac… - … of Physiology-Cell …, 2024 - journals.physiology.org
Extranuclear localization of long non-coding RNAs (lncRNAs) is poorly understood. Based
on machine learning evaluations, we propose a lncRNA-mitochondrial interaction pathway …