Animal Models of Coenzyme Q Deficiency: Mechanistic and Translational Learnings

Coenzyme Q (CoQ) is a vital lipophilic molecule that is endogenously synthesized in the mitochondria of each cell. The CoQ biosynthetic pathway is complex and not completely characterized, and it involves at least thirteen catalytic and regulatory proteins. Once it is synthesized, CoQ exerts a wide...

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Autores principales:	Pilar González-García, Eliana Barriocanal-Casado, María Elena Díaz-Casado, Sergio López-Herrador, Agustín Hidalgo-Gutiérrez, Luis C. López
Formato:	article
Lenguaje:	EN
Publicado:	MDPI AG 2021
Materias:	preclinical models pathomechanisms therapy mitochondria mitochondrial disease bioenergetics Therapeutics. Pharmacology RM1-950
Acceso en línea:	https://doaj.org/article/a1125c1a52ef4f1eb8439aceaba38194
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spelling	oai:doaj.org-article:a1125c1a52ef4f1eb8439aceaba381942021-11-25T16:26:11ZAnimal Models of Coenzyme Q Deficiency: Mechanistic and Translational Learnings10.3390/antiox101116872076-3921https://doaj.org/article/a1125c1a52ef4f1eb8439aceaba381942021-10-01T00:00:00Zhttps://www.mdpi.com/2076-3921/10/11/1687https://doaj.org/toc/2076-3921Coenzyme Q (CoQ) is a vital lipophilic molecule that is endogenously synthesized in the mitochondria of each cell. The CoQ biosynthetic pathway is complex and not completely characterized, and it involves at least thirteen catalytic and regulatory proteins. Once it is synthesized, CoQ exerts a wide variety of mitochondrial and extramitochondrial functions thank to its redox capacity and its lipophilicity. Thus, low levels of CoQ cause diseases with heterogeneous clinical symptoms, which are not always understood. The decreased levels of CoQ may be primary caused by defects in the CoQ biosynthetic pathway or secondarily associated with other diseases. In both cases, the pathomechanisms are related to the CoQ functions, although further experimental evidence is required to establish this association. The conventional treatment for CoQ deficiencies is the high doses of oral CoQ<sub>10</sub> supplementation, but this therapy is not effective for some specific clinical presentations, especially in those involving the nervous system. To better understand the CoQ biosynthetic pathway, the biological functions linked to CoQ and the pathomechanisms of CoQ deficiencies, and to improve the therapeutic outcomes of this syndrome, a variety of animal models have been generated and characterized in the last decade. In this review, we show all the animal models available, remarking on the most important outcomes that each model has provided. Finally, we also comment some gaps and future research directions related to CoQ metabolism and how the current and novel animal models may help in the development of future research studies.Pilar González-GarcíaEliana Barriocanal-CasadoMaría Elena Díaz-CasadoSergio López-HerradorAgustín Hidalgo-GutiérrezLuis C. LópezMDPI AGarticlepreclinical modelspathomechanismstherapymitochondriamitochondrial diseasebioenergeticsTherapeutics. PharmacologyRM1-950ENAntioxidants, Vol 10, Iss 1687, p 1687 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	preclinical models pathomechanisms therapy mitochondria mitochondrial disease bioenergetics Therapeutics. Pharmacology RM1-950
spellingShingle	preclinical models pathomechanisms therapy mitochondria mitochondrial disease bioenergetics Therapeutics. Pharmacology RM1-950 Pilar González-García Eliana Barriocanal-Casado María Elena Díaz-Casado Sergio López-Herrador Agustín Hidalgo-Gutiérrez Luis C. López Animal Models of Coenzyme Q Deficiency: Mechanistic and Translational Learnings
description	Coenzyme Q (CoQ) is a vital lipophilic molecule that is endogenously synthesized in the mitochondria of each cell. The CoQ biosynthetic pathway is complex and not completely characterized, and it involves at least thirteen catalytic and regulatory proteins. Once it is synthesized, CoQ exerts a wide variety of mitochondrial and extramitochondrial functions thank to its redox capacity and its lipophilicity. Thus, low levels of CoQ cause diseases with heterogeneous clinical symptoms, which are not always understood. The decreased levels of CoQ may be primary caused by defects in the CoQ biosynthetic pathway or secondarily associated with other diseases. In both cases, the pathomechanisms are related to the CoQ functions, although further experimental evidence is required to establish this association. The conventional treatment for CoQ deficiencies is the high doses of oral CoQ<sub>10</sub> supplementation, but this therapy is not effective for some specific clinical presentations, especially in those involving the nervous system. To better understand the CoQ biosynthetic pathway, the biological functions linked to CoQ and the pathomechanisms of CoQ deficiencies, and to improve the therapeutic outcomes of this syndrome, a variety of animal models have been generated and characterized in the last decade. In this review, we show all the animal models available, remarking on the most important outcomes that each model has provided. Finally, we also comment some gaps and future research directions related to CoQ metabolism and how the current and novel animal models may help in the development of future research studies.
format	article
author	Pilar González-García Eliana Barriocanal-Casado María Elena Díaz-Casado Sergio López-Herrador Agustín Hidalgo-Gutiérrez Luis C. López
author_facet	Pilar González-García Eliana Barriocanal-Casado María Elena Díaz-Casado Sergio López-Herrador Agustín Hidalgo-Gutiérrez Luis C. López
author_sort	Pilar González-García
title	Animal Models of Coenzyme Q Deficiency: Mechanistic and Translational Learnings
title_short	Animal Models of Coenzyme Q Deficiency: Mechanistic and Translational Learnings
title_full	Animal Models of Coenzyme Q Deficiency: Mechanistic and Translational Learnings
title_fullStr	Animal Models of Coenzyme Q Deficiency: Mechanistic and Translational Learnings
title_full_unstemmed	Animal Models of Coenzyme Q Deficiency: Mechanistic and Translational Learnings
title_sort	animal models of coenzyme q deficiency: mechanistic and translational learnings
publisher	MDPI AG
publishDate	2021
url	https://doaj.org/article/a1125c1a52ef4f1eb8439aceaba38194
work_keys_str_mv	AT pilargonzalezgarcia animalmodelsofcoenzymeqdeficiencymechanisticandtranslationallearnings AT elianabarriocanalcasado animalmodelsofcoenzymeqdeficiencymechanisticandtranslationallearnings AT mariaelenadiazcasado animalmodelsofcoenzymeqdeficiencymechanisticandtranslationallearnings AT sergiolopezherrador animalmodelsofcoenzymeqdeficiencymechanisticandtranslationallearnings AT agustinhidalgogutierrez animalmodelsofcoenzymeqdeficiencymechanisticandtranslationallearnings AT luisclopez animalmodelsofcoenzymeqdeficiencymechanisticandtranslationallearnings
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Animal Models of Coenzyme Q Deficiency: Mechanistic and Translational Learnings

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