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 …
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 …
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 …
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
Neuropsychiatric disorders have complex pathophysiological constructs, requiring
translational models to improve our understanding thereof. Mitochondrial dysfunction …
translational models to improve our understanding thereof. Mitochondrial dysfunction …
Exploring thermal sensitivities and adaptations of oxidative phosphorylation pathways
Temperature shifts are a major challenge to animals; they drive adaptations in organisms
and species, and affect all physiological functions in ectothermic organisms. Understanding …
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 …
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
Mitochondria are dynamic multitask organelles that function as hubs for many metabolic
pathways. They produce most ATP via the oxidative phosphorylation pathway, a critical …
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
Background Elevated plasma levels of alpha‐aminoadipic acid (2‐AAA) have been
associated with the development of type 2 diabetes and atherosclerosis. However, the …
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
Mitochondrial diseases (MD), such as Leigh syndrome (LS), present with severe
neurological and muscular phenotypes in patients, but have no known cure and limited …
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 …
phosphorylation system, currently lack curative treatments. Human-relevant, high-throughput …