Biochemistry II - Glycogen Storage Disease (GSD) Already Passed Review Exam what are glycogen storage diseases? - ANS-class of more than 10 distinct disorders of glycogen metabolism. they can result in either an an increase or decrease in amount of glycogen why do GSDs mainly affect the liver and the muscle? - ANS-these are the main storage sites of glycogen. other tissues like the kidney can also be affected what is GSD type I? - ANS-type I glycogenesis: deficiency in glucose-6-phosphatase (G6Pase) what is the cori cycle? - ANS-the metabolic pathway in which lactate produced by anaerobic glycolysis in the muscles moves to the liver and is converted to glucose, which then returns to the muscles and is metabolized back to lactate what is G6Pase? - ANS-enzyme involved in the terminal step of both glycogen breakdown (glycogenolysis) and gluconeogenesis (formation of glucose from non-glucose precursors e.g. pyruvate or lactate). this means G6Pase is critically important in the regulation of blood glucose levels what reaction is catalysed by G6Pase? - ANS-G6Pase catalyses the hydrolysis of G6P to glucose and inorganic phosphate (Pi): G6P + H2O --> glucose + Pi tissue-specific location of G6Pase: in which 3 tissues is the enzyme located? - ANS-1
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