The effects of L-alanine supplementation in late-onset glycogen storage disease type II

Myopathy in glycogen storage disease type II (GSD-II) is slowly progressive . Five subjects with the late-onset form of GSD-II (age range, 15 to 47 yea rs) and seven healthy control subjects (age range, 28 to 55 years) were stu died. Following alanine supplementation, resting energy expenditure decreas ed in patients with GSD-II (p < 0.05) compared to values seen in control su bjects. Leucine flux decreased (p < 0.004), as did leucine oxidation, to le vels lower than those observed in control subjects (p < 0.001). L-Alanine r educes protein turnover and catabolism in GSD-II.