Patients with mitochondrial myopathies (MM) or myophosphorylase deficiency (McArdle's
disease, McA) show impaired capacity for O2 extraction, low maximal aerobic power, and …

Purpose The aim was to identify additional noninvasive tools allowing to detect and to
quantify the metabolic impairment in patients with mitochondrial myopathies (MM) or …

Exercise intolerance is one of the most common symptoms in patients with mitochondrial
myopathies (MM). At the whole body level, this is characterized by a reduction in maximal …

Cycle exercise has repeatedly been used to diagnose patients suspected of having
mitochondrial myopathy (MM), in whom exercise intolerance and lactic acidosis are …

Metabolic myopathies due to a variety of enzymatic deficiencies are well recognized. The
dynamics of oxygen delivery and utilization during exercise have not been observed …

Bioenergetic sufficiency can be quantitatively assayed by nuclear magnetic resonance
spectroscopy (MRS) and on a relative basis by tissue optical spectroscopy (NIRS). Nuclear …

Clinical phenotypes of persons with mitochondrial DNA (mtDNA) mutations vary
considerably. Therefore, diagnosing mitochondrial myopathy (MM) patients can be …

Impaired skeletal muscle oxidative phosphorylation in patients with severe mitochondrial
respiratory chain defects results in disabling exercise intolerance that is associated with a …

Abstract 31 Phosphorus nuclear magnetic resonance spectroscopy was used to examine
skeletal muscle in 29 patients with mitochondrial myopathy, 9 male and 20 female …

Skeletal muscle oxidative capacity is highly plastic, strongly associated with whole body
aerobic capacity (16, 18) and state of health. Loss of muscle oxidative capacity is associated …