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22. A 20-year-old man reports painful cramps during exercise. The cramps are usually followed by a red tint in his urine. Consumption of glucose-containing electrolyte- replacement drinks before exercising does not alleviate the symptoms. Additional tests indicate that he has a mild case of hemolytic anemia. The patient most likely has a deficiency of which enzyme?
A. Branching enzyme
B. Glucose-6-phosphatase
C. α-Glucosidase
D. Liver phosphorylase
E. Phosphofructokinase
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E. Phosphofructokinase, type 7 glycogen storage disease (Tarui disease)= muscle cramping + myoglobinuria.
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Thanks my q bank posted all as wrong so I wasnt sure which is right? Never heard if type 7
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it should be Muscle Phosphorylase deficiency Type 5 McArdle's Disease,,but its not in options ,,,so whats ur Q bank says Somber?
Phosphofructokinase is rate limiting step in Glycolysis,,there is no Type 7 disease i heard so far.
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yeah,, this link tells the type 7 GSD..due to deficiency of Phophofructokinase.I dont know baout it before.Thanks fox.
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you are right fox.thanks.
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Hi Pmle, do you have electronic Stedman medical dictionary? Under GCD, there is a table listed all the GCDs, including some of those not mentioned in Kaplan notes. But I think those will be very unlikely to appear in the real exam.
