The Complexity of Making Ubiquinone.
ER–mitochondria contact sites
coenzyme Q
mitochondrial dysfunction
ubiquinone
ubiquinone biosynthesis
Journal
Trends in endocrinology and metabolism: TEM
ISSN: 1879-3061
Titre abrégé: Trends Endocrinol Metab
Pays: United States
ID NLM: 9001516
Informations de publication
Date de publication:
12 2019
12 2019
Historique:
received:
03
06
2019
revised:
19
08
2019
accepted:
20
08
2019
pubmed:
12
10
2019
medline:
25
8
2020
entrez:
12
10
2019
Statut:
ppublish
Résumé
Ubiquinone (UQ, coenzyme Q) is an essential electron transfer lipid in the mitochondrial respiratory chain. It is a main source of mitochondrial reactive oxygen species (ROS) but also has antioxidant properties. This mix of characteristics is why ubiquinone supplementation is considered a potential therapy for many diseases involving mitochondrial dysfunction. Mutations in the ubiquinone biosynthetic pathway are increasingly being identified in patients. Furthermore, secondary ubiquinone deficiency is a common finding associated with mitochondrial disorders and might exacerbate these conditions. Recent developments have suggested that ubiquinone biosynthesis occurs in discrete domains of the mitochondrial inner membrane close to ER-mitochondria contact sites. This spatial requirement for ubiquinone biosynthesis could be the link between secondary ubiquinone deficiency and mitochondrial dysfunction, which commonly results in loss of mitochondrial structural integrity.
Identifiants
pubmed: 31601461
pii: S1043-2760(19)30176-6
doi: 10.1016/j.tem.2019.08.009
pii:
doi:
Substances chimiques
Reactive Oxygen Species
0
Ubiquinone
1339-63-5
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
929-943Subventions
Organisme : CIHR
ID : FDN-159916
Pays : Canada
Informations de copyright
Copyright © 2019 Elsevier Ltd. All rights reserved.