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Glucose --> glucose-6-phosphate
69%
317/462
Glucose-6-phosphate --> fructose-6-phosphate
8%
39/462
Fructose-6-phosphate --> fructose-1,6-biphosphate
14%
65/462
Glyceraldehyde-3-phosphate --> 1,3-bisphosphoglycerate
3%
13/462
Phosphoenolpyruvate --> pyruvate
4%
18/462
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MODY 2 results from a defective glucokinase. Glucokinase is an enzyme that traps glucose inside the cell by converting it to glucose-6-phophate. MODY 2 is an autosomal dominant, monogenic form of diabetes that results from any number of mutations on the glucokinase gene on chromosome 7. Normally glucokinase functions to trap glucose within the cell by converting it to glucose-6-phosphate, which does not have a transporter to exit the cell. Glucokinase allows the cell to sense the amount of glucose in the body and normally triggers insulin release. Since the defective glucokinase does not trigger the release of insulin at 90 mg/dL but works at a much higher level > 126 mg/dL, the individual experiences a chronic, mild, asymptomatic hyperglycemia. Incorrect Answers: These enzymes are not involved in glucose sensing but are involved in glycolysis. Answer 2: Phosphoglucose isomerase is involved in isomerizing glucose-6-phosphate to fructose-6-phosphate. Answer 3: Phosphofructokinase is involved in adding a phosphate group to fructose-6-phosphate producing fructose-1,6-biphosphate. Answer 4: Phosphoglycerate kinase is involved in adding a phosphate group to glyceraldehyde-3-phosphate producing 1,3-bisphosphoglycerate. Answer 5: Pyruvate kinase is involved in removing a phosphate group from phosphoenolpyruvate producing pyruvate while generating 1 molecule of ATP in the process.
3.8
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