Abstract：
:Mitochondrial disorders (MDs) are caused by impairment of the mitochondrial electron transport chain (ETC). The ETC is needed for oxidative phosphorylation, which provides the cell with the most efficient energy outcome in terms of ATP production. One of the pathogenic mechanisms of MDs is the accumulation of reactive oxygen species. Mitochondrial dysfunction and oxidative stress appear to also have a strong impact on the pathogenesis of neurodegenerative diseases and cancer. The treatment of MDs is still inadequate. Therapies that have been attempted include ETC cofactors, other metabolites secondarily decreased in MDs, antioxidants, and agents acting on lactic acidosis. However, the role of these dietary supplements in the treatment of the majority of MDs remains unclear. This article reviews the rationale for their use and their role in clinical practice in the context of MDs and other disorders involving mitochondrial dysfunction.
journal_name
Nutr Revjournal_title
Nutrition reviewsauthors
Orsucci D,Filosto M,Siciliano G,Mancuso Mdoi
10.1111/j.1753-4887.2009.00221.xsubject
Has Abstractpub_date
2009-08-01 00:00:00pages
427-38issue
8eissn
0029-6643issn
1753-4887pii
NURE221journal_volume
67pub_type
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