The assembly, regulation and function of the mitochondrial respiratory chain

I Vercellino, LA Sazanov - Nature reviews Molecular cell biology, 2022 - nature.com
The mitochondrial oxidative phosphorylation system is central to cellular metabolism. It
comprises five enzymatic complexes and two mobile electron carriers that work in a …

[HTML][HTML] Mitochondrial complex I ROS production and redox signaling in hypoxia

CN Okoye, SA Koren, AP Wojtovich - Redox biology, 2023 - Elsevier
Mitochondria are a main source of cellular energy. Oxidative phosphorylation (OXPHOS) is
the major process of aerobic respiration. Enzyme complexes of the electron transport chain …

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) …

[HTML][HTML] Autophagy promotes cell survival by maintaining NAD levels

T Kataura, L Sedlackova, EG Otten, R Kumari… - Developmental cell, 2022 - cell.com
Autophagy is an essential catabolic process that promotes the clearance of surplus or
damaged intracellular components. Loss of autophagy in age-related human pathologies …

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 …

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 …

Cooperative assembly of the mitochondrial respiratory chain

E Fernández-Vizarra, C Ugalde - Trends in Biochemical Sciences, 2022 - cell.com
Deep understanding of the pathophysiological role of the mitochondrial respiratory chain
(MRC) relies on a well-grounded model explaining how its biogenesis is regulated. The lack …

Mitochondrial complex I as a pathologic and therapeutic target for Parkinson's disease

KP Tryphena, US Nikhil, P Pinjala… - ACS Chemical …, 2023 - ACS Publications
The prevalence of Parkinson's disease (PD) continues to increase despite substantial
research. Mounting evidence states that dysfunctional mitochondrial bioenergetics play a …

Insights into complex I assembly: function of NDUFAF1 and a link with cardiolipin remodeling

J Schiller, E Laube, I Wittig, W Kühlbrandt, J Vonck… - Science …, 2022 - science.org
Respiratory complex I is a~ 1-MDa proton pump in mitochondria. Its structure has been
revealed in great detail, but the structural basis of its assembly, in humans involving at least …

Dynamics of human mitochondrial complex I assembly: implications for neurodegenerative diseases

G Giachin, R Bouverot, S Acajjaoui… - Frontiers in molecular …, 2016 - frontiersin.org
Neurons are extremely energy demanding cells and highly dependent on the mitochondrial
oxidative phosphorylation (OXPHOS) system. Mitochondria generate the energetic potential …