title: ATP, phosphocreatine and lactate in exercising muscle in mitochondrial disease and McArdle's disease.
abstract: We studied exercise-induced changes in the adenosine triphosphate (ATP), phosphocreatine (PCr), and lactate levels in the skeletal muscle of mitochondrial patients and patients with McArdle's disease. Needle muscle biopsy specimens for biochemical measurement were obtained before and immediately after maximal short-term bicycle exercise test from 12 patients suffering from autosomal dominant and recessive forms of progressive external ophthalmoplegia and multiple deletions of mitochondrial DNA (adPEO, arPEO, respectively), five patients with mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) 3243 A-->G point mutation, and four patients with McArdle's disease. Muscle ATP and PCr levels at rest or after exercise did not differ significantly from those of the controls in any patient group. In patients with mitochondrial disease, muscle lactate tended to be lower at rest and increase more during exercise than in controls, the most remarkable rise being measured in patients with adPEO with generalized muscle symptoms and in patients with MELAS point mutation. In McArdle patients, the muscle lactate level decreased during exercise. No correlation was found between the muscle ATP and PCr levels and the respiratory chain enzyme activity.
authors.affiliation: Institute of Neurosciences, Department of Neurology, Helsinki University Hospital, 00029 HUS, Helsinki, Finland. mervi.lofberg@hus.fi
authors.forename: M
authors.initials: M
authors.lastname: Löfberg
authors.forename: H
authors.initials: H
authors.lastname: Lindholm
authors.forename: H
authors.initials: H
authors.lastname: Näveri
authors.forename: A
authors.initials: A
authors.lastname: Majander
authors.forename: A
authors.initials: A
authors.lastname: Suomalainen
authors.forename: A
authors.initials: A
authors.lastname: Paetau
authors.forename: A
authors.initials: A
authors.lastname: Sovijärvi
authors.forename: M
authors.initials: M
authors.lastname: Härkönen
authors.forename: H
authors.initials: H
authors.lastname: Somer
mesh_terms: D000255:Adenosine Triphosphate / Q000378:metabolism*; D000328:Adult; D000368:Aged; D004272:DNA, Mitochondrial / Q000235:genetics; D004579:Electron Transport / Q000502:physiology; D004798:Enzymes / Q000378:metabolism; D015444:Exercise / Q000502:physiology*; D005080:Exercise Test; D017353:Gene Deletion; D005799:Genes, Dominant; D005808:Genes, Recessive; D006012:Glycogen Storage Disease Type V / Q000378:metabolism / Q000503:physiopathology*; D006801:Humans; D019344:Lactic Acid / Q000378:metabolism*; D017241:MELAS Syndrome / Q000378:metabolism / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D017240:Mitochondrial Myopathies / Q000378:metabolism / Q000503:physiopathology*; D018482:Muscle, Skeletal / Q000378:metabolism*; D009886:Ophthalmoplegia / Q000235:genetics / Q000378:metabolism / Q000503:physiopathology; D010725:Phosphocreatine / Q000378:metabolism*; D010809:Physical Fitness
chemical_list: D004272:DNA, Mitochondrial; D004798:Enzymes; D010725:Phosphocreatine; D019344:Lactic Acid; D000255:Adenosine Triphosphate