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Biochemistry Glycogen Metabolism and Glycogen Storage 1ebdaebb

McArdles disease is due to the deficiency of:

A
Glucose 1 phosphatase
B
Glucose 1, 6 diphosphatase
C
Glucose 6 phosphatase
D
Myophosphorylase
High-Yield Explanation
Ans: D (Myophosphorylase) Ref: Harper's illustrated Biochemistry,28th edition,Chapter 19,pp 160Explanation:McArdles diseaseType V Glycogen storage diseaseDeficiency: muscle phosphorylase/myophosp- horylasePremature chain termination, incorrect folding etc can cause loss of catalytic activitySkeletal muscle is affectedExercise-induced muscular pain, cramps, progressive weakness, myoglobinuriaNo increase in blood lactate after exerciseGlycogen storage disordersDisorderAffected TissueEnzyme defectType 0LiverGlycogensynthaseType I; Von Gierke'sLiver, kidney, intestineGlucose-6-phosphataseType II; Pompe'sAilLysosomal alpha glucosidaseType III; Forbe'sLiver, muscle, heartGlycogendebranching enzymeType IV; Anderson'sLiverBranching enzymeType V; Mc Ardle'sMusclesmuscle phosphory laseType VI; Her'sLiverLiver phosphon/laseType VII; Tauri'sSkeletal muscle, RBCMuscle and Liver PFK

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