Coenzyme Q at the Hinge of Health and Metabolic Diseases

Coenzyme Q is a unique lipidic molecule highly conserved in evolution and essential to maintaining aerobic metabolism. It is endogenously synthesized in all cells by a very complex pathway involving a group of nuclear genes that share high homology among species. This pathway is tightly regulated at...

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Autores principales: Juan Diego Hernández-Camacho, Laura García-Corzo, Daniel José Moreno Fernández-Ayala, Plácido Navas, Guillermo López-Lluch
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Publicado: MDPI AG 2021
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Acceso en línea:https://doaj.org/article/7de4b7ffdbc147d5ac911a3a99b42286
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spelling oai:doaj.org-article:7de4b7ffdbc147d5ac911a3a99b422862021-11-25T16:28:34ZCoenzyme Q at the Hinge of Health and Metabolic Diseases10.3390/antiox101117852076-3921https://doaj.org/article/7de4b7ffdbc147d5ac911a3a99b422862021-11-01T00:00:00Zhttps://www.mdpi.com/2076-3921/10/11/1785https://doaj.org/toc/2076-3921Coenzyme Q is a unique lipidic molecule highly conserved in evolution and essential to maintaining aerobic metabolism. It is endogenously synthesized in all cells by a very complex pathway involving a group of nuclear genes that share high homology among species. This pathway is tightly regulated at transcription and translation, but also by environment and energy requirements. Here, we review how coenzyme Q reacts within mitochondria to promote ATP synthesis and also integrates a plethora of metabolic pathways and regulates mitochondrial oxidative stress. Coenzyme Q is also located in all cellular membranes and plasma lipoproteins in which it exerts antioxidant function, and its reaction with different extramitochondrial oxidoreductases contributes to regulate the cellular redox homeostasis and cytosolic oxidative stress, providing a key factor in controlling various apoptosis mechanisms. Coenzyme Q levels can be decreased in humans by defects in the biosynthesis pathway or by mitochondrial or cytosolic dysfunctions, leading to a highly heterogeneous group of mitochondrial diseases included in the coenzyme Q deficiency syndrome. We also review the importance of coenzyme Q levels and its reactions involved in aging and age-associated metabolic disorders, and how the strategy of its supplementation has had benefits for combating these diseases and for physical performance in aging.Juan Diego Hernández-CamachoLaura García-CorzoDaniel José Moreno Fernández-AyalaPlácido NavasGuillermo López-LluchMDPI AGarticlecoenzyme Qubiquinonemetabolic diseaserare diseasemitochondriaTherapeutics. PharmacologyRM1-950ENAntioxidants, Vol 10, Iss 1785, p 1785 (2021)
institution DOAJ
collection DOAJ
language EN
topic coenzyme Q
ubiquinone
metabolic disease
rare disease
mitochondria
Therapeutics. Pharmacology
RM1-950
spellingShingle coenzyme Q
ubiquinone
metabolic disease
rare disease
mitochondria
Therapeutics. Pharmacology
RM1-950
Juan Diego Hernández-Camacho
Laura García-Corzo
Daniel José Moreno Fernández-Ayala
Plácido Navas
Guillermo López-Lluch
Coenzyme Q at the Hinge of Health and Metabolic Diseases
description Coenzyme Q is a unique lipidic molecule highly conserved in evolution and essential to maintaining aerobic metabolism. It is endogenously synthesized in all cells by a very complex pathway involving a group of nuclear genes that share high homology among species. This pathway is tightly regulated at transcription and translation, but also by environment and energy requirements. Here, we review how coenzyme Q reacts within mitochondria to promote ATP synthesis and also integrates a plethora of metabolic pathways and regulates mitochondrial oxidative stress. Coenzyme Q is also located in all cellular membranes and plasma lipoproteins in which it exerts antioxidant function, and its reaction with different extramitochondrial oxidoreductases contributes to regulate the cellular redox homeostasis and cytosolic oxidative stress, providing a key factor in controlling various apoptosis mechanisms. Coenzyme Q levels can be decreased in humans by defects in the biosynthesis pathway or by mitochondrial or cytosolic dysfunctions, leading to a highly heterogeneous group of mitochondrial diseases included in the coenzyme Q deficiency syndrome. We also review the importance of coenzyme Q levels and its reactions involved in aging and age-associated metabolic disorders, and how the strategy of its supplementation has had benefits for combating these diseases and for physical performance in aging.
format article
author Juan Diego Hernández-Camacho
Laura García-Corzo
Daniel José Moreno Fernández-Ayala
Plácido Navas
Guillermo López-Lluch
author_facet Juan Diego Hernández-Camacho
Laura García-Corzo
Daniel José Moreno Fernández-Ayala
Plácido Navas
Guillermo López-Lluch
author_sort Juan Diego Hernández-Camacho
title Coenzyme Q at the Hinge of Health and Metabolic Diseases
title_short Coenzyme Q at the Hinge of Health and Metabolic Diseases
title_full Coenzyme Q at the Hinge of Health and Metabolic Diseases
title_fullStr Coenzyme Q at the Hinge of Health and Metabolic Diseases
title_full_unstemmed Coenzyme Q at the Hinge of Health and Metabolic Diseases
title_sort coenzyme q at the hinge of health and metabolic diseases
publisher MDPI AG
publishDate 2021
url https://doaj.org/article/7de4b7ffdbc147d5ac911a3a99b42286
work_keys_str_mv AT juandiegohernandezcamacho coenzymeqatthehingeofhealthandmetabolicdiseases
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AT danieljosemorenofernandezayala coenzymeqatthehingeofhealthandmetabolicdiseases
AT placidonavas coenzymeqatthehingeofhealthandmetabolicdiseases
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