About this Research Topic
Metabolic myopathies (MM) and motor neuron diseases (MND) comprise a heterogeneous group of disorders characterized by impaired oxidative metabolism and/or reduced muscle strength. Patients affected by MM and MND show signs of progressive reduction of exercise tolerance and easy fatigability, with a progressive and devasting effect on the quality of life. The complex multifactorial pathogenesis of MM and MND, from cellular to systems derangement perspective, is an extraordinary challenge for human physiology research in order to improve the resiliency of this patients, in childhood and adult life, or to accompany them till the end of their lives.
Finding a cure or taking care of patients affected by the wide clinical spectrum of MM and MND requires the recognition of mechanisms that jeopardize both oxidative metabolism as well as skeletal muscle strength during exercise. Indeed, at rest, in a state where there is no activity, oxidative metabolic resources and muscle strength are essentially underused. On the other hand, the full expression of integration and efficiency of our body systems is critically exposed during exercise. The incapacity of the cells to breakdown carbohydrates and fatty acids to generate adenosine triphosphate (as in MM) and/or the inability of motor neurons to activate functional firing motor units (as in MND) are the result of the inadequacy of cellular and body systems to cope with exercise requirements. In MM and MND, exercise triggers the inherent flaws in cellular energy metabolism and motor units’ recruitment due to the diseases and finally reveal a severely impaired physical function.
The aim of this Topic is to encourage further research in the area and foster a prolific discussion from different perspectives. Thus, it seems rational to evaluate the efficiency of the O2 transport-utilization chain during exercise to recognize either early manifestation of oxidative metabolism impairment and residual oxidative capacity. Accordingly, the assessment of the impact of precision training and/or diet, “as medicine”, can lead to new therapies aimed at counteracting the conditions threatening oxidative metabolism and the determinants of force in skeletal muscle during exercise.
The proposed Research Topic “Strategies to fight exercise intolerance in neuromuscular disorders” will address relevant issues from different perspectives including investigation of oxidative metabolism and muscle strength during exercise, as well as precision exercise and/or diet used as therapy in patients. We encourage submission of all manuscripts ranging from cell metabolism to integrative physiology during exercise in patients with MM and MND including, but not limited, to: cardiopulmonary O2 delivery to tissues; skeletal muscles O2 extraction (counting mitochondrial function); exercise tolerance and muscle strength after personalized exercise and/or dietary regimen; strategies to introduce precision exercise and/or diet in clinical settings.
We welcome original research papers, narrative or systematic review contributions and editorial articles. Submissions from clinicians, exercise physiologists, molecular biologists, geneticists, nutritionists and dietitians are encouraged.
Some potential themes of interest for this Research Topic will be:
Functional evaluation of skeletal muscle oxidative metabolism in patients with MM and MND, including VO2 uptake and Near Infrared Spectroscopy investigations;
Effects of precision exercise training in patients with MM and MND, including the evaluation of the impact of different exercise regimens (intensities, resistance and/or endurance, interval training, etc) as well as the adaptation to exercise training programs from the molecular and cellular level to the integrated response of systems;
Effect of training in animal models of MM and MND, including new biomarkers enlighten early parameters of muscle adaptations/remodelling induced by various exercise regimens or acute response to exercise;
Impact of diet on mitochondrial function in animal models of MM and MND, including the response to hypoxia induced by exercise;
Impact of diet on oxidative metabolism during exercise in patients with MM and MND, including clinical evaluations and hearth and muscle imaging;
Correlation between clinical predictors of MM and MND progression and exercise physiology outcomes of reduced exercise tolerance;
Strategies to introduce exercise and/or diet as medicine into practical clinical applications or practice in systems supporting care of patients with MM and MND; and
Impact of precision exercise and/or diet to the quality of life of patients with MM and MND.
Keywords: Oxidative metabolism, motor neuron disorders, metabolic myopathies, exercise, diet
Important Note: All contributions to this Research Topic must be within the scope of the section and journal to which they are submitted, as defined in their mission statements. Frontiers reserves the right to guide an out-of-scope manuscript to a more suitable section or journal at any stage of peer review.