Mitochondrial disorders of the OXPHOS system
E Fernandez‐Vizarra, M Zeviani - FEBS letters, 2021 - Wiley Online Library
Mitochondrial disorders are among the most frequent inborn errors of metabolism, their
primary cause being the dysfunction of the oxidative phosphorylation system (OXPHOS) …
primary cause being the dysfunction of the oxidative phosphorylation system (OXPHOS) …
Mitochondrial structure and bioenergetics in normal and disease conditions
M Protasoni, M Zeviani - International journal of molecular sciences, 2021 - mdpi.com
Mitochondria are ubiquitous intracellular organelles found in almost all eukaryotes and
involved in various aspects of cellular life, with a primary role in energy production. The …
involved in various aspects of cellular life, with a primary role in energy production. The …
Assembly of mammalian oxidative phosphorylation complexes I–V and supercomplexes
A Signes, E Fernandez-Vizarra - Essays in biochemistry, 2018 - portlandpress.com
The assembly of the five oxidative phosphorylation system (OXPHOS) complexes in the
inner mitochondrial membrane is an intricate process. The human enzymes comprise core …
inner mitochondrial membrane is an intricate process. The human enzymes comprise core …
Mitochondrial OXPHOS biogenesis: co-regulation of protein synthesis, import, and assembly pathways
JX Tang, K Thompson, RW Taylor… - International Journal of …, 2020 - mdpi.com
The assembly of mitochondrial oxidative phosphorylation (OXPHOS) complexes is an
intricate process, which—given their dual-genetic control—requires tight co-regulation of two …
intricate process, which—given their dual-genetic control—requires tight co-regulation of two …
Cysteine toxicity drives age-related mitochondrial decline by altering iron homeostasis
Mitochondria and lysosomes are functionally linked, and their interdependent decline is a
hallmark of aging and disease. Despite the long-standing connection between these …
hallmark of aging and disease. Despite the long-standing connection between these …
[HTML][HTML] Mitochondrial microproteins link metabolic cues to respiratory chain biogenesis
Electron transport chain (ETC) biogenesis is tightly coupled to energy levels and availability
of ETC subunits. Complex III (CIII), controlling ubiquinol: ubiquinone ratio in ETC, is an …
of ETC subunits. Complex III (CIII), controlling ubiquinol: ubiquinone ratio in ETC, is an …
The mitochondrial acyl carrier protein (ACP) coordinates mitochondrial fatty acid synthesis with iron sulfur cluster biogenesis
Mitochondrial fatty acid synthesis (FASII) and iron sulfur cluster (FeS) biogenesis are both
vital biosynthetic processes within mitochondria. In this study, we demonstrate that the …
vital biosynthetic processes within mitochondria. In this study, we demonstrate that the …
Nuclear gene mutations as the cause of mitochondrial complex III deficiency
E Fernández-Vizarra, M Zeviani - Frontiers in genetics, 2015 - frontiersin.org
Complex III (CIII) deficiency is one of the least common oxidative phosphorylation defects
associated to mitochondrial disease. CIII constitutes the center of the mitochondrial …
associated to mitochondrial disease. CIII constitutes the center of the mitochondrial …
Yeast as a system for modeling mitochondrial disease mechanisms and discovering therapies
Mitochondrial diseases are severe and largely untreatable. Owing to the many essential
processes carried out by mitochondria and the complex cellular systems that support these …
processes carried out by mitochondria and the complex cellular systems that support these …
ACP acylation is an acetyl-CoA-dependent modification required for electron transport chain assembly
JG Van Vranken, SM Nowinski, KJ Clowers, MY Jeong… - Molecular cell, 2018 - cell.com
The electron transport chain (ETC) is an important participant in cellular energy conversion,
but its biogenesis presents the cell with numerous challenges. To address these …
but its biogenesis presents the cell with numerous challenges. To address these …