Fat metabolism during exercise in patients with mitochondrial disease

Tina Dysgaard Jeppesen; Mette Cathrine Orngreen; Gerrit Van Hall; Ronald G Haller; John Vissing

doi:10.1001/archneurol.2009.24

Fat metabolism during exercise in patients with mitochondrial disease

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

Exercise and Muscle

Publikation: Bidrag til tidsskrift › Tidsskriftartikel › Forskning › peer review

16 Citationer (Scopus)

Abstract

Udgivelsesdato: 2009-Mar

Originalsprog	Engelsk
Tidsskrift	Archives of Neurology
Vol/bind	66
Udgave nummer	3
Sider (fra-til)	365-70
Antal sider	5
ISSN	0003-9942
DOI	https://doi.org/10.1001/archneurol.2009.24
Status	Udgivet - 2009

Bibliografisk note

Keywords: Adult; Carbohydrate Metabolism; Case-Control Studies; Electron Transport; Epinephrine; Exercise; Fatty Acids; Female; Glucose; Hormones; Humans; Insulin; Lipid Metabolism; Male; Middle Aged; Mitochondrial Diseases; Muscle, Skeletal; Norepinephrine; Oxygen Consumption; Palmitic Acid

Adgang til dokumentet

10.1001/archneurol.2009.24

Citationsformater

@article{c15a3e404f6d11de87b8000ea68e967b,

title = "Fat metabolism during exercise in patients with mitochondrial disease",

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.",

author = "Jeppesen, {Tina Dysgaard} and Orngreen, {Mette Cathrine} and {Van Hall}, Gerrit and Haller, {Ronald G} and John Vissing",

note = "Keywords: Adult; Carbohydrate Metabolism; Case-Control Studies; Electron Transport; Epinephrine; Exercise; Fatty Acids; Female; Glucose; Hormones; Humans; Insulin; Lipid Metabolism; Male; Middle Aged; Mitochondrial Diseases; Muscle, Skeletal; Norepinephrine; Oxygen Consumption; Palmitic Acid",

year = "2009",

doi = "10.1001/archneurol.2009.24",

language = "English",

volume = "66",

pages = "365--70",

journal = "Archives of Neurology",

issn = "0003-9942",

publisher = "The JAMA Network",

number = "3",

}

TY - JOUR

T1 - Fat metabolism during exercise in patients with mitochondrial disease

AU - Jeppesen, Tina Dysgaard

AU - Orngreen, Mette Cathrine

AU - Van Hall, Gerrit

AU - Haller, Ronald G

AU - Vissing, John

N1 - Keywords: Adult; Carbohydrate Metabolism; Case-Control Studies; Electron Transport; Epinephrine; Exercise; Fatty Acids; Female; Glucose; Hormones; Humans; Insulin; Lipid Metabolism; Male; Middle Aged; Mitochondrial Diseases; Muscle, Skeletal; Norepinephrine; Oxygen Consumption; Palmitic Acid

PY - 2009

Y1 - 2009

N2 - 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.

AB - 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.

U2 - 10.1001/archneurol.2009.24

DO - 10.1001/archneurol.2009.24

M3 - Journal article

C2 - 19273755

SN - 0003-9942

VL - 66

SP - 365

EP - 370

JO - Archives of Neurology

JF - Archives of Neurology

IS - 3

ER -