A 16-year-old female is recently diagnosed with a deficiency of muscle glycogen phosphorylase. She and her family are concerned and ask the following questions. Based on what you know about skeletal muscle glycogenolysis and muscle metabolism, please respond to each question with a thoughtful answer that describes the metabolism in these various scenarios in language that your patient will understand, while also being sure to include the role of the relevant metabolic pathways, specific enzymes, and stimulus for their activity: I’m not sure I understand the issue. Can you explain how muscle glycogen is used normally during exercise? I really like to take long walks; will Istill be able to do this with my friends? As a result of this deficiency, will I need to get up at night to eat to maintain my blood glucose levels? Do I need to worry about producing excessive lactate during intense anaerobic exercise?
Electron Transport Chain
The electron transport chain, also known as the electron transport system, is a group of proteins that transfer electrons through a membrane within mitochondria to create a gradient of protons that drives adenosine triphosphate (ATP)synthesis. The cell uses ATP as an energy source for metabolic processes and cellular functions. ETC involves series of reactions that convert redox energy from NADH (nicotinamide adenine dinucleotide (NAD) + hydrogen (H)) and FADH2(flavin adenine dinucleotide (FAD)) oxidation into proton-motive force(PMF), which is then used to synthesize ATP through conformational changes in the ATP synthase complex, a process known as oxidative phosphorylation.
Metabolism
Picture a campfire. It keeps the body warm on a cold night and provides light. To ensure that the fire keeps burning, fuel needs to be added(pieces of wood in this case). When a small piece is added, the fire burns bright for a bit and then dies down unless more wood is added. But, if too many pieces are placed at a time, the fire escalates and burns for a longer time, without actually burning away all the pieces that have been added. Many of them, especially the larger chunks or damp pieces, remain unburnt.
Cellular Respiration
Cellular respiration is the cellular process involved in the generation of adenosine triphosphate (ATP) molecules from the organic nutritional source obtained from the diet. It is a universal process observed in all types of life forms. The glucose (chemical formula C6H12O6) molecules are the preferred raw material for cell respiration as it possesses a simple structure and is highly efficient in nature.
A 16-year-old female is recently diagnosed with a deficiency of muscle glycogen phosphorylase. She and her family are concerned and ask the following questions. Based on what you know about skeletal muscle glycogenolysis and muscle
- I’m not sure I understand the issue. Can you explain how muscle glycogen is used normally during exercise?
- I really like to take long walks; will Istill be able to do this with my friends?
- As a result of this deficiency, will I need to get up at night to eat to maintain my blood glucose levels?
- Do I need to worry about producing excessive lactate during intense anaerobic exercise?
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