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| Name | Class |
|---|---|
| Oslo University Hospital | OTHER |
| University of Southern Denmark | OTHER |
| University of Copenhagen | OTHER |
| Maastricht University |
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The purpose of this study is to investigate the response after one bout of moderate-heavy resistance exercise in patients suffering from Myotonic Dystrophy type 1. There is still doubt about if these patients could benefit from resistance exercise, or if this mode of exercise is detrimental to their mobility and health. We aim to monitor the subjects during the recovery phase and investigate recovery in several ways.
Myotonic dystrophy type 1 (DM1) is an autosomal, dominantly inherited, muscular disease and the most common muscular dystrophy amongst adults. The disease is characterized by progressive myopathy and myotonia, first evident in the distal parts of the body: calves and forearms, further progressing towards proximal parts. A recent review by Voet and co-workers (2013) concludes that exercise is safe for DM1-patients, and based on our current knowledge, DM1-patients should be able to gain positive adaptations from exercise. However, the physiological outcomes of an exercise stimulus are not well studied in this population and because of alterations in important Ca2+ handling systems the recovery processes might be significantly different from healthy muscles.
The aim of this study is to investigate the acute responses to moderate-heavy resistance exercise in DM1-patients. We aim to establish a timeline for recovery of muscle strength, measured as maximal voluntary contraction. In line with this, we want to investigate if any muscular damage has occurred with both direct and indirect measures, and possible mechanisms leading to muscle damage, i.e. impaired Ca2+ handling.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| DM1-Ex | Experimental | Moderate-heavy resistance exercise of one leg in DM1-patients |
|
| DM1-Rest | No Intervention | Control leg (i.e. no exercise) in DM1-patients | |
| Ctrl-Ex | Experimental | Moderate-heavy resistance exercise of one leg in healthy participants |
|
| Ctrl-Rest | No Intervention | Control leg (i.e. no exercise) in healthy participants |
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Moderate-Heavy Resistance Exercise | Other | Leg press: 3 x 8 repetitions at 75% of 1 repetition maximum, 3 minutes rest between sets. Knee extension: 3 x 6 repetitions at 85% of 1 repetition maximum, 3 minutes rest between sets. |
| Measure | Description | Time Frame |
|---|---|---|
| Change in muscle strength | Recovery of knee extension torque | Baseline, and 5 minutes, 3 hours, 24 hours, 48 hours, and 71 hours after exercise |
| Measure | Description | Time Frame |
|---|---|---|
| Muscle protein synthesis rate | Rate of muscle protein synthesis in m. vastus lateralis between baseline and 27 hours after final session of resistance exercise | Baseline and 27 hours after final session of resistance exercise |
| Muscle protein breakdown rate |
| Measure | Description | Time Frame |
|---|---|---|
| Change in muscle damage | Myofibrillar disruption and necrosis observed in muscle tissue samples from m. vastus lateralis with electron microscopy | Baseline and 27 hours after final session of resistance exercise |
| Change in muscle regeneration |
Inclusion Criteria:
Exclusion Criteria:
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| Name | Affiliation | Role |
|---|---|---|
| Truls Raastad, PhD | Norwegian School of Sport Sciences | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Norwegian School of Sport Sciences | Oslo | 0863 | Norway |
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| ID | Term |
|---|---|
| D009223 | Myotonic Dystrophy |
| ID | Term |
|---|---|
| D009136 | Muscular Dystrophies |
| D020966 | Muscular Disorders, Atrophic |
| D009135 | Muscular Diseases |
| D009140 | Musculoskeletal Diseases |
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| OTHER |
| University of Oslo | OTHER |
Participants in both groups (patients and control) perform unilateral leg resistance exercise, contralateral leg serves as control
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Rate of muscle protein breakdown in m. vastus lateralis between baseline and 27 hours after final session of resistance exercise |
| Baseline and 27 hours after final session of resistance exercise |
Appearance of macrophages, satellite cell localization, and disruption of membrane in single muscle fibers and cross-sections using confocal microscopy
| Baseline and 27 hours after final session of resistance exercise |
| Change in calcium cycling | Calcium cycling in muscle tissue samples from m. vastus lateralis | Baseline and 27 hours after final session of resistance exercise |
| Change in contractile function | Assessment of contractile function in single muscle fibers from m. vastus lateralis | Baseline and 27 hours after final session of resistance exercise |
| Change in calcium-related protein abundances | Levels of protein and phosphorylation status using Western blotting | Baseline and 27 hours after final session of resistance exercise |
| Muscle fiber type | Fiber type composition in samples from m. vastus lateralis | Baseline |
| Muscle fiber type | Fiber type composition in samples from m. vastus lateralis | 27 hours after final session of resistance exercise |
| Change in heat shock proteins | Localization and expression of heat shock proteins using Western blotting and Immunohistochemistry | Baseline and 27 hours after final session of resistance exercise |
| Change in anabolic signaling | Expression and phosphorylation status of central mediators in anabolic signaling pathways using Western blotting | Baseline and 27 hours after final session of resistance exercise |
| Change in autophagy | Expression and phosphorylation status of central mediators in pathways regulating muscle protein breakdown using Western blotting | Baseline and 27 hours after final session of resistance exercise |
| Change in biomarkers of muscle damage | Levels of serum Creatine kinase, Myoglobin, and Troponin I | Baseline, and 3 hours, 24 hours, 48 hours, 71 hours, and 96 hours after exercise |
| Change in biomarker of muscle damage | Level of titin-N fragment in urine | Baseline, and 20 hours, 44 hours, 68 hours, and 92 hours after exercise |
| Change in Fatigue | Electrical stimulation of m. vastus lateralis and m. vastus medialis at 10, 20, 50, and 80 Hz | Baseline, and 1 hour after exercise |
| Change in muscle stiffness | Muscle stiffness measured with shear wave elastography in static and dynamic conditions | Baseline, and 50 minutes after exercise |
| Change in muscle soreness | Subjective rating of muscle soreness using a visual analogue scale (0-10) | Baseline, and 3 hours, 24 hours, 48 hours, and 71 hours after exercise |
| Change in muscle swelling | Thickness of m. vastus lateralis using ultrasound B-mode | Baseline, and 3 hours, 24 hours, 48 hours, and 71 hours after exercise |
| D020967 | Myotonic Disorders |
| D020271 | Heredodegenerative Disorders, Nervous System |
| D019636 | Neurodegenerative Diseases |
| D009422 | Nervous System Diseases |
| D009468 | Neuromuscular Diseases |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |