Mcardle’s Disease is a type of glycogen storage disease (also known as glycogenosis or dextrinosis). Glycogen storage disease is the consequence of the deformity of the human body to synthesize glycogen in the liver, or to break glycogen down within the liver and muscles. There are two main types of glycogen storage disease one is genetic GSD (or congenital metabolic disease), which is caused by the defect of genes that do not code the enzymes needed for the synthesis or the conversion of the substrate and this causes the accumulation of toxic substances in the body which interfere with the normal function of the body; the second type of GSD is acquired, it is caused by the impairment of the nitrogen based castanospermine (inhibits glucosidase
…show more content…
Exercises with isometric muscle contractions (for example lifting weights and gripping exercises) have a high demand of energy from a typically small muscle mass and also increase the pressure on the muscle thus making the usually abundant supply of oxygenated blood cut off (nature.com). This is when the patient’s muscles solely rely on anaerobic respiration to supply energy for the activity they are doing, however anaerobic respiration is supplied from the glycogen stores in the muscle’s cells (the oxygenated blood is cut off, thus the glucose is also cut off from the working muscle, so glycogen is used). Dynamic or aerobic exercises involve the movement and contraction of a large muscle mass but with a small pressure on the muscle (for example, climbing or running up the stairs, running, and fast walking), this type of exercise also triggers exercise intolerance. This occurs because a patient with Mcardle’s disease has the inability to produce the molecule pyruvate, which is essential for the function of the Krebs Cycle in aerobic respiration. This then causes the reduced rate of the oxidative phosphorylation process of aerobic respiration (which happens in the electron transport chain). This is reflected in the patients by the low concentration of ATP and a decrease in the muscle cell phosphorylation potential (medicineplus.gov) which would also lead to the accumulation of inorganic phosphate and ADP and the inhibition of the enzyme ATP Synthase, and inorganic molecule pump reactions. The inability of these reactions happening results in the early muscle fatigue and contractures
Exercises with isometric muscle contractions (for example lifting weights and gripping exercises) have a high demand of energy from a typically small muscle mass and also increase the pressure on the muscle thus making the usually abundant supply of oxygenated blood cut off (nature.com). This is when the patient’s muscles solely rely on anaerobic respiration to supply energy for the activity they are doing, however anaerobic respiration is supplied from the glycogen stores in the muscle’s cells (the oxygenated blood is cut off, thus the glucose is also cut off from the working muscle, so glycogen is used). Dynamic or aerobic exercises involve the movement and contraction of a large muscle mass but with a small pressure on the muscle (for example, climbing or running up the stairs, running, and fast walking), this type of exercise also triggers exercise intolerance. This occurs because a patient with Mcardle’s disease has the inability to produce the molecule pyruvate, which is essential for the function of the Krebs Cycle in aerobic respiration. This then causes the reduced rate of the oxidative phosphorylation process of aerobic respiration (which happens in the electron transport chain). This is reflected in the patients by the low concentration of ATP and a decrease in the muscle cell phosphorylation potential (medicineplus.gov) which would also lead to the accumulation of inorganic phosphate and ADP and the inhibition of the enzyme ATP Synthase, and inorganic molecule pump reactions. The inability of these reactions happening results in the early muscle fatigue and contractures