Fat metabolism during exercise in patients with mitochondrial disease

Tina Dysgaard Jeppesen, Mette Cathrine Ørngreen, Gerrit Van Hall, Ronald G. Haller, John Vissing

Research output: Contribution to journalArticle

9 Citations (Scopus)

Abstract

Objective: To determine whether patients with defects of the respiratory chain have metabolic adaptations that promote a preferential use of fats or carbohydrates, similar to what is observed in metabolic myopathies affecting glycolysis or fat oxidation. Design: Causation and case-control study. Fat metabolism was determined by means of indirect calorimetry and stable isotope technique in patients and healthy subjects. Patients carried various types and loads (mean [SE], 72% [5%]) of mitochondrial DNA (mtDNA) mutations in skeletal muscle. All subjects exercised at the same absolute workload (mean [SE], 65 [10] W), corresponding to 72% (in patients) and 30% (in healthy subjects) of maximum oxygen consumption. Setting: Neuromuscular research unit. Participants: Ten patients with mtDNA mutations and 10 sex-matched healthy subjects. Main Outcome Measures: Fat turnover, plasma concentrations of palmitate and total free fatty acids, glucose mobilization, and total carbohydrate oxidation. Results: Fat turnover and plasma concentrations of palmitate and total free fatty acids were similar in patients and healthy subjects at rest and during exercise. In line with the higher relative workload of the patients, glucose mobilization and total carbohydrate oxidation were higher in the patients compared with the healthy subjects. Conclusion: During moderate-intensity exercise, the balance between fat and carbohydrate use in patients with mtDNA mutations matches that seen in healthy subjects, indicating that manipulating dietary fat and carbohydrate content is not a feasible therapeutic option to improve exercise intolerance in these disorders.

Original languageEnglish (US)
Pages (from-to)365-370
Number of pages6
JournalArchives of Neurology
Volume66
Issue number3
DOIs
StatePublished - Mar 2009

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Mitochondrial Diseases
Fats
Exercise
Healthy Volunteers
Mitochondrial DNA
Carbohydrates
Palmitates
Workload
Nonesterified Fatty Acids
Mutation
Dietary Carbohydrates
Fat
Metabolism
Glucose
Indirect Calorimetry
Dietary Fats
Glycolysis
Muscular Diseases
Electron Transport
Oxygen Consumption

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Fat metabolism during exercise in patients with mitochondrial disease. / Jeppesen, Tina Dysgaard; Ørngreen, Mette Cathrine; Van Hall, Gerrit; Haller, Ronald G.; Vissing, John.

In: Archives of Neurology, Vol. 66, No. 3, 03.2009, p. 365-370.

Research output: Contribution to journalArticle

Jeppesen, Tina Dysgaard ; Ørngreen, Mette Cathrine ; Van Hall, Gerrit ; Haller, Ronald G. ; Vissing, John. / Fat metabolism during exercise in patients with mitochondrial disease. In: Archives of Neurology. 2009 ; Vol. 66, No. 3. pp. 365-370.
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