The mitochondrial coenzyme Q junction and complex III: Biochemistry and pathophysiology

R Banerjee, J Purhonen, J Kallijärvi - The FEBS journal, 2022 - Wiley Online Library
Coenzyme Q (CoQ, ubiquinone) is the electron‐carrying lipid in the mitochondrial electron
transport system (ETS). In mammals, it serves as the electron acceptor for nine mitochondrial …

Ndufs4 knockout mouse models of Leigh syndrome: pathophysiology and intervention

MAE Van De Wal, MJW Adjobo-Hermans, J Keijer… - Brain, 2022 - academic.oup.com
Mitochondria are small cellular constituents that generate cellular energy (ATP) by oxidative
phosphorylation (OXPHOS). Dysfunction of these organelles is linked to a heterogeneous …

Impaired mitochondrial complex I function as a candidate driver in the biological stress response and a concomitant stress-induced brain metabolic reprogramming in …

TL Emmerzaal, G Preston, B Geenen, V Verweij… - Translational …, 2020 - nature.com
Mitochondria play a critical role in bioenergetics, enabling stress adaptation, and therefore,
are central in biological stress responses and stress-related complex psychopathologies. To …

Reviewing the mitochondrial dysfunction paradigm in rodent models as platforms for neuropsychiatric disease research

DJ van Rensburg, Z Lindeque, BH Harvey, SF Steyn - Mitochondrion, 2022 - Elsevier
Neuropsychiatric disorders have complex pathophysiological constructs, requiring
translational models to improve our understanding thereof. Mitochondrial dysfunction …

Exploring thermal sensitivities and adaptations of oxidative phosphorylation pathways

H Lemieux, PU Blier - Metabolites, 2022 - mdpi.com
Temperature shifts are a major challenge to animals; they drive adaptations in organisms
and species, and affect all physiological functions in ectothermic organisms. Understanding …

Genetically predisposed and resilient animal models of depression reveal divergent responses to early-life adversity

AJ Whitney, Z Lindeque, R Kruger… - Acta neuropsychiatrica, 2024 - cambridge.org
Objective: Early-life adversity (ELA) is one of the strongest predictors of childhood
depression that may be exacerbated by a genetic predisposition to develop depression. We …

Clinical insights into mitochondrial neurodevelopmental and neurodegenerative disorders: their biosignatures from mass spectrometry-based metabolomics

H Li, M Uittenbogaard, L Hao, A Chiaramello - Metabolites, 2021 - mdpi.com
Mitochondria are dynamic multitask organelles that function as hubs for many metabolic
pathways. They produce most ATP via the oxidative phosphorylation pathway, a critical …

Genetic Architecture of Plasma Alpha‐Aminoadipic Acid Reveals a Relationship With High‐Density Lipoprotein Cholesterol

M Shi, C Wang, H Mei, M Temprosa… - Journal of the …, 2022 - Am Heart Assoc
Background Elevated plasma levels of alpha‐aminoadipic acid (2‐AAA) have been
associated with the development of type 2 diabetes and atherosclerosis. However, the …

Metallothionein 1 Overexpression Does Not Protect Against Mitochondrial Disease Pathology in Ndufs4 Knockout Mice

HC Miller, R Louw, M Mereis, G Venter, JD Boshoff… - Molecular …, 2021 - Springer
Mitochondrial diseases (MD), such as Leigh syndrome (LS), present with severe
neurological and muscular phenotypes in patients, but have no known cure and limited …

[HTML][HTML] CRISPR-Cas9 mediated knockout of NDUFS4 in human iPSCs: A model for mitochondrial complex I deficiency

S Goolab, K Terburgh, C du Plessis… - … et Biophysica Acta (BBA …, 2025 - Elsevier
Mitochondrial diseases, often caused by defects in complex I (CI) of the oxidative
phosphorylation system, currently lack curative treatments. Human-relevant, high-throughput …