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What is glycogen storage illness? Glycogen storage diseases (GSDs) are a group of rare conditions in which your physique can’t use or store glycogen correctly. They’re sorts of inherited (handed from dad or mum to child) metabolic disorders. Advertising on our site helps help our mission. We don't endorse non-Cleveland Clinic services or products. Glycogen is the stored form of glucose (sugar). Glucose is your body’s essential source of vitality. It comes from carbohydrates (a macronutrient) in certain foods and fluids you devour. When your body doesn’t instantly need glucose for energy, it stores glucose primarily in your skeletal muscles and liver as glycogen for later use. Your physique creates glycogen from glucose via a process called glycogenesis. When your physique needs extra gas, it breaks down glycogen to be used by way of a course of known as glycogenolysis. Several enzymes are chargeable for these two processes. Glycogen storage diseases happen whenever you don’t have one or more of those enzymes. Your body can’t use stored glycogen for power or maintain steady boost blood flow naturally glucose ranges. This can cause a number of issues, together with frequent symptomatic low blood sugar (hypoglycemia), liver injury and muscle weakness. Researchers know extra about some varieties than others. GSD largely impacts your liver or muscles. Some sorts cause issues in different areas of your body, as effectively. For each kind of GSD, there’s a lack (deficiency) of a sure enzyme concerned in glycogen storage or breakdown. Healthcare suppliers may refer to each kind primarily based on the specific enzyme that’s lacking or the scientist who found that kind of GSD. Glycogen storage illness is uncommon. GSD kind I (von Gierke disease), the commonest type, happens in roughly 1 in 100,000 births. |
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