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...
| Main Authors: | , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
MDPI AG
2021-10-01
|
| Series: | Antioxidants |
| Subjects: | |
| Online Access: | https://www.mdpi.com/2076-3921/10/11/1687 |
| _version_ | 1797511446432579584 |
|---|---|
| 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 |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-03-10T05:45:23Z |
| format | Article |
| id | doaj.art-a1125c1a52ef4f1eb8439aceaba38194 |
| institution | Directory Open Access Journal |
| issn | 2076-3921 |
| language | English |
| last_indexed | 2024-03-10T05:45:23Z |
| publishDate | 2021-10-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Antioxidants |
| spelling | doaj.art-a1125c1a52ef4f1eb8439aceaba381942023-11-22T22:12:13ZengMDPI AGAntioxidants2076-39212021-10-011011168710.3390/antiox10111687Animal Models of Coenzyme Q Deficiency: Mechanistic and Translational LearningsPilar González-García0Eliana Barriocanal-Casado1María Elena Díaz-Casado2Sergio López-Herrador3Agustín Hidalgo-Gutiérrez4Luis C. López5Departamento de Fisiología, Facultad de Medicina, Universidad de Granada, 18016 Granada, SpainDepartamento de Fisiología, Facultad de Medicina, Universidad de Granada, 18016 Granada, SpainDepartamento de Fisiología, Facultad de Medicina, Universidad de Granada, 18016 Granada, SpainDepartamento de Fisiología, Facultad de Medicina, Universidad de Granada, 18016 Granada, SpainDepartamento de Fisiología, Facultad de Medicina, Universidad de Granada, 18016 Granada, SpainDepartamento de Fisiología, Facultad de Medicina, Universidad de Granada, 18016 Granada, SpainCoenzyme 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.https://www.mdpi.com/2076-3921/10/11/1687preclinical modelspathomechanismstherapymitochondriamitochondrial diseasebioenergetics |
| spellingShingle | 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 Antioxidants preclinical models pathomechanisms therapy mitochondria mitochondrial disease bioenergetics |
| title | 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_short | Animal Models of Coenzyme Q Deficiency: Mechanistic and Translational Learnings |
| title_sort | animal models of coenzyme q deficiency mechanistic and translational learnings |
| topic | preclinical models pathomechanisms therapy mitochondria mitochondrial disease bioenergetics |
| url | https://www.mdpi.com/2076-3921/10/11/1687 |
| work_keys_str_mv | AT pilargonzalezgarcia animalmodelsofcoenzymeqdeficiencymechanisticandtranslationallearnings AT elianabarriocanalcasado animalmodelsofcoenzymeqdeficiencymechanisticandtranslationallearnings AT mariaelenadiazcasado animalmodelsofcoenzymeqdeficiencymechanisticandtranslationallearnings AT sergiolopezherrador animalmodelsofcoenzymeqdeficiencymechanisticandtranslationallearnings AT agustinhidalgogutierrez animalmodelsofcoenzymeqdeficiencymechanisticandtranslationallearnings AT luisclopez animalmodelsofcoenzymeqdeficiencymechanisticandtranslationallearnings |