title: Nutritional and exercise-based therapies in the treatment of mitochondrial disease.
abstract: OBJECTIVE
  This review will critically summarize the nutritional and exercise-based interventions that have been used to treat mitochondrial disease, with a focus on the biochemical or molecular rationale for their use as well as recent advances in the field.
  
  
  RESULTS
  Many nutritional-based treatment strategies have been used in an attempt to target energy impairment and its sequelae. Recently, coenzyme Q10, idebenone and triacylglycerol have been shown to bypass defective respiratory enzymes or scavenge free radicals, whereas creatine monohydrate has provided an alternative energy source. Thiamine has been used to decrease lactate levels and increase flux through aerobic metabolism, and riboflavin has been used as a precursor to complexes I and II. Several therapies employing various antioxidants in combination with other supplements have been effective at targeting several of the final common pathways of mitochondrial disease. Miscellaneous supplements, such as L-arginine and uridine, have also had recent success. However, although positive responses have been reported with these agents, many reports have shown no benefit, and there is widespread disparity in the literature. An alternative approach to treatment is exercise training. Both resistance and endurance exercise training have had positive outcomes in patients with mitochondrial disease, although several questions remain to be answered.
  
  
  CONCLUSIONS
  There is no currently recognized treatment for mitochondrial disease. Future clinical trials are needed, as well as research into the potential for in-vitro screening of various compounds within affected cells from patients. Until this time, an accurate diagnosis will facilitate treatment on a case-by-case basis.
authors.affiliation: Department of Medical Sciences, McMaster University, Hamilton, Ontario, Canada.
authors.forename: Douglas J
authors.initials: DJ
authors.lastname: Mahoney
authors.forename: Gianni
authors.initials: G
authors.lastname: Parise
authors.forename: Mark A
authors.initials: MA
authors.lastname: Tarnopolsky
mesh_terms: D004734:Energy Metabolism; D005081:Exercise Therapy; D006801:Humans; D028361:Mitochondrial Diseases / Q000178:diet therapy* / Q000235:genetics / Q000378:metabolism / Q000628:therapy*; D010085:Oxidative Phosphorylation; D010807:Physical Endurance; D017382:Reactive Oxygen Species; D016896:Treatment Outcome
chemical_list: D017382:Reactive Oxygen Species