The potential of mitochondrial genome engineering

P Silva-Pinheiro, M Minczuk - Nature Reviews Genetics, 2022 - nature.com
Mitochondria are subject to unique genetic control by both nuclear DNA and their own
genome, mitochondrial DNA (mtDNA), of which each mitochondrion contains multiple …

Yeast mitochondria: an overview of mitochondrial biology and the potential of mitochondrial systems biology

C Malina, C Larsson, J Nielsen - FEMS yeast research, 2018 - academic.oup.com
Mitochondria are dynamic organelles of endosymbiotic origin that are essential components
of eukaryal cells. They contain their own genetic machinery, have multicopy genomes and …

[HTML][HTML] An in vitro system to silence mitochondrial gene expression

LD Cruz-Zaragoza, S Dennerlein, A Linden, R Yousefi… - Cell, 2021 - cell.com
The human mitochondrial genome encodes thirteen core subunits of the oxidative
phosphorylation system, and defects in mitochondrial gene expression lead to severe …

Site-specific CRISPR-based mitochondrial DNA manipulation is limited by gRNA import

L Schmiderer, D Yudovich, L Oburoglu, M Hjort… - Scientific Reports, 2022 - nature.com
Achieving CRISPR Cas9-based manipulation of mitochondrial DNA (mtDNA) has been a
long-standing goal and would be of great relevance for disease modeling and for clinical …

Moving towards clinical trials for mitochondrial diseases

RDS Pitceathly, N Keshavan… - Journal of inherited …, 2021 - Wiley Online Library
Primary mitochondrial diseases represent some of the most common and severe inherited
metabolic disorders, affecting~ 1 in 4,300 live births. The clinical and molecular diversity …

Mitochondrial genome evolution in yeasts: an all-encompassing view

KC Freel, A Friedrich, J Schacherer - FEMS yeast research, 2015 - academic.oup.com
Mitochondria are important organelles that harbor their own genomes encoding a key set of
proteins that ensure respiration and provide the eukaryotic cell with energy. Recent …

Gene therapy for mitochondrial diseases: Current status and future perspective

A Di Donfrancesco, G Massaro, I Di Meo, V Tiranti… - Pharmaceutics, 2022 - mdpi.com
Mitochondrial diseases (MDs) are a group of severe genetic disorders caused by mutations
in the nuclear or mitochondrial genome encoding proteins involved in the oxidative …

Modular assembly of yeast mitochondrial ATP synthase

M Rak, S Gokova, A Tzagoloff - The EMBO journal, 2011 - embopress.org
The mitochondrial ATP synthase (F1–F0 complex) of Saccharomces cerevisiae is a
composite of different structural and functional units that jointly couple ATP synthesis and …

The Cbp3–Cbp6 complex coordinates cytochrome b synthesis with bc1 complex assembly in yeast mitochondria

S Gruschke, K Römpler, M Hildenbeutel… - Journal of Cell …, 2012 - rupress.org
Respiratory chain complexes in mitochondria are assembled from subunits derived from two
genetic systems. For example, the bc1 complex consists of nine nuclear encoded subunits …

Cbp3–Cbp6 interacts with the yeast mitochondrial ribosomal tunnel exit and promotes cytochrome b synthesis and assembly

S Gruschke, K Kehrein, K Römpler, K Gröne… - Journal of Cell …, 2011 - rupress.org
Mitochondria contain their own genetic system to express a small number of hydrophobic
polypeptides, including cytochrome b, an essential subunit of the bc1 complex of the …