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Biosynthesis, Deficiency, and Supplementation of Coenzyme Q.
Staiano, Carmine; García-Corzo, Laura; Mantle, David; Turton, Nadia; Millichap, Lauren E; Brea-Calvo, Gloria; Hargreaves, Iain.
Afiliación
  • Staiano C; Centro Andaluz de Biología del Desarrollo, Universidad Pablo de Olavide-CSIC-JA, 41013 Sevilla, Spain.
  • García-Corzo L; Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER), Instituto de Salud Carlos III, 28029 Madrid, Spain.
  • Mantle D; Departamento de Fisiología, Anatomía y Biología Celular, Universidad Pablo de Olavide, 41013 Sevilla, Spain.
  • Turton N; Centro Andaluz de Biología del Desarrollo, Universidad Pablo de Olavide-CSIC-JA, 41013 Sevilla, Spain.
  • Millichap LE; Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER), Instituto de Salud Carlos III, 28029 Madrid, Spain.
  • Brea-Calvo G; Departamento de Fisiología, Anatomía y Biología Celular, Universidad Pablo de Olavide, 41013 Sevilla, Spain.
  • Hargreaves I; Pharma Nord (UK) Ltd., Morpeth NE61 2DB, UK.
Antioxidants (Basel) ; 12(7)2023 Jul 21.
Article en En | MEDLINE | ID: mdl-37508007
Originally identified as a key component of the mitochondrial respiratory chain, Coenzyme Q (CoQ or CoQ10 for human tissues) has recently been revealed to be essential for many different redox processes, not only in the mitochondria, but elsewhere within other cellular membrane types. Cells rely on endogenous CoQ biosynthesis, and defects in this still-not-completely understood pathway result in primary CoQ deficiencies, a group of conditions biochemically characterised by decreased tissue CoQ levels, which in turn are linked to functional defects. Secondary CoQ deficiencies may result from a wide variety of cellular dysfunctions not directly linked to primary synthesis. In this article, we review the current knowledge on CoQ biosynthesis, the defects leading to diminished CoQ10 levels in human tissues and their associated clinical manifestations.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Idioma: En Revista: Antioxidants (Basel) Año: 2023 Tipo del documento: Article País de afiliación: España Pais de publicación: Suiza

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Idioma: En Revista: Antioxidants (Basel) Año: 2023 Tipo del documento: Article País de afiliación: España Pais de publicación: Suiza