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] Coenzyme Q10 Supplementation in Aging and Disease

JD Hernández-Camacho, M Bernier… - Frontiers in …, 2018 - frontiersin.org
Coenzyme Q (CoQ) is an essential component of the mitochondrial electron transport chain
and an antioxidant in plasma membranes and lipoproteins. It is endogenously produced in …

[HTML][HTML] Mitochondria regulate intracellular coenzyme Q transport and ferroptotic resistance via STARD7

S Deshwal, M Onishi, T Tatsuta, T Bartsch, E Cors… - Nature cell …, 2023 - nature.com
Coenzyme Q (or ubiquinone) is a redox-active lipid that serves as universal electron carrier
in the mitochondrial respiratory chain and antioxidant in the plasma membrane limiting lipid …

[HTML][HTML] Disorders of human coenzyme Q10 metabolism: an overview

I Hargreaves, RA Heaton, D Mantle - International journal of molecular …, 2020 - mdpi.com
Coenzyme Q10 (CoQ10) has a number of vital functions in all cells, both mitochondrial and
extramitochondrial. In addition to its key role in mitochondrial oxidative phosphorylation …

Mitochondrial dysfunction in inherited renal disease and acute kidney injury

F Emma, G Montini, SM Parikh, L Salviati - Nature Reviews Nephrology, 2016 - nature.com
Mitochondria are increasingly recognized as key players in genetic and acquired renal
diseases. Most mitochondrial cytopathies that cause renal symptoms are characterized by …

[HTML][HTML] Coenzyme Q biosynthesis in health and disease

MJ Acosta, LV Fonseca, MA Desbats, C Cerqua… - … et Biophysica Acta (BBA …, 2016 - Elsevier
Coenzyme Q (CoQ, or ubiquinone) is a remarkable lipid that plays an essential role in
mitochondria as an electron shuttle between complexes I and II of the respiratory chain, and …

[HTML][HTML] Mitochondria related cell death modalities and disease

C Tian, Y Liu, Z Li, P Zhu, M Zhao - Frontiers in Cell and …, 2022 - frontiersin.org
Mitochondria are well known as the centre of energy metabolism in eukaryotic cells.
However, they can not only generate ATP through the tricarboxylic acid cycle and oxidative …

[HTML][HTML] Mitochondrial cardiomyopathies

AW El-Hattab, F Scaglia - Frontiers in Cardiovascular Medicine, 2016 - frontiersin.org
Mitochondria are found in all nucleated human cells and perform various essential functions,
including the generation of cellular energy. Mitochondria are under dual genome control …

Genetic bases and clinical manifestations of coenzyme Q10 (CoQ10) deficiency

MA Desbats, G Lunardi, M Doimo, E Trevisson… - Journal of inherited …, 2015 - Springer
Coenzyme Q 10 is a remarkable lipid involved in many cellular processes such as energy
production through the mitochondrial respiratory chain (RC), beta-oxidation of fatty acids …

[HTML][HTML] Coenzyme Q10 supplementation–In ageing and disease

J Aaseth, J Alexander, U Alehagen - Mechanisms of Ageing and …, 2021 - Elsevier
Abstract Coenzyme Q 10 (CoQ 10) is an essential component of the mitochondrial electron
transport chain. It is also an antioxidant in cellular membranes and lipoproteins. All cells …