Brain Advance Access originally published online on June 30, 2006
Brain 2006 129(12):3402-3412; doi:10.1093/brain/awl149
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Aerobic training is safe and improves exercise capacity in patients with mitochondrial myopathy
1 Department of Neurology, Neuromuscular Research Unit, The Copenhagen Muscle Research Centre Rigshospitalet, Copenhagen, Denmark 2 Department of Clinical Genetics, National University Hospital Rigshospitalet, Copenhagen, Denmark
Correspondence to: Tina Dysgaard Jeppesen, Neuromuscular Research Unit, section 7611, National University Hospital, Rigshospitalet, Blegdamsvej 9, DK-2100 Copenhagen, Denmark E-mail: dysgaard{at}rh.dk
Exercise intolerance is a prominent symptom in patients with mitochondrial myopathy (MM), but it is still unsettled whether exercise training is safe and beneficial for patients with MM. To address this, we studied the effect of 12 weeks cycle training on exercise capacity, quality of life and underlying molecular and cellular events in five patients with single large-scale deletions, one with a microdeletion and 14 with point mutations of mitochondrial DNA (mtDNA), and 13 healthy subjects. Each training session lasted 30 min, and was performed at an intensity of 70% of VO2max (maximal oxygen uptake). Each subject performed 50 training sessions in 12 weeks. All subjects were evaluated before and after training, and 13 MM patients were studied after 8 weeks of deconditioning. Evaluation included VO2max and mutation load and mtDNA quantity, mitochondrial enzymatic activity, and number of centrally nucleated, apoptotic, ragged red and cytochrome oxidase (COX)-negative fibres in muscle biopsies from the quadriceps muscle. After 12 weeks of training, VO2max and muscle citrate synthase increased in MM (26 and 67%) and healthy (17 and 65%) subjects, while mtDNA quantity in muscle only increased in the MM patients (81%). In the MM patients, training did not change mtDNA mutation load in muscle, mitochondrial enzyme complex activities, muscle morphology and plasma creatine kinase. After deconditioning, VO2max and citrate synthase activity returned to values before training, while muscle mtDNA mutation load decreased. These findings show that aerobic training efficiently improves oxidative capacity in MM patients. Based on unchanged levels of mutant load in muscle, morphological findings on muscle biopsy and plasma creatine kinase levels during training, the treatment appears to be safe. Regular, supervised aerobic exercise is therefore recommended in MM patients with the studied mutations.
Key Words: mitochondrial myopathy; training; mtDNA; mutation load
Abbreviations: CK, creatine kinase; HR, heart rate; MM, mitochondrial myopathy; mtDNA, mitochondrial DNA; RRF, ragged red fibres; VO2max, maximal oxygen uptake; Wmax, maximal workload
Received February 9, 2006. Revised April 27, 2006. Accepted May 3, 2006.
CiteULike 
Connotea 
Del.icio.us What's this?
This article has been cited by other articles:
|
|
 |
|
  T. Wenz, F. Diaz, D. Hernandez, and C. T. Moraes Endurance exercise is protective for mice with mitochondrial myopathy J Appl Physiol, May 1, 2009; 106(5): 1712 - 1719. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 N. Preisler, G. Andersen, F. Thogersen, C. Crone, T. D. Jeppesen, F. Wibrand, and J. Vissing Effect of aerobic training in patients with spinal and bulbar muscular atrophy (Kennedy disease) Neurology, January 27, 2009; 72(4): 317 - 323. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. L. Murphy, E. L. Blakely, A. M. Schaefer, L. He, P. Wyrick, R. G. Haller, R. W. Taylor, D. M. Turnbull, and T. Taivassalo Resistance training in patients with single, large-scale deletions of mitochondrial DNA Brain, November 1, 2008; 131(11): 2832 - 2840. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 T Higashimoto, E E Baldwin, J I Gold, and R G Boles Reflex sympathetic dystrophy: complex regional pain syndrome type I in children with mitochondrial disease and maternal inheritance Arch. Dis. Child., May 1, 2008; 93(5): 390 - 397. [Abstract] [Full Text] [PDF]
|
|