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 years) and seven healthy control subjects (age range, 28 to 55 years) were studied. Following alanine supplementation, resting energy expenditure decreased in patients with GSD-II (p < 0.05) compared to values seen in control subjects. Leucine flux decreased (p < 0.004), as did leucine oxidation, to levels lower than those observed in control subjects (p < 0.001). l-Alanine reduces protein turnover and catabolism in GSD-II.
Bodamer
O A
OA
Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA. obodamer@bcm.tmc.edu
Halliday
D
D
Leonard
J V
JV
D000293:Adolescent; D000328:Adult; D000409:Alanine / Q000008:administration & dosage*; D004734:Energy Metabolism; D005260:Female; D005947:Glucose / Q000378:metabolism; D006009:Glycogen Storage Disease Type II / Q000188:drug therapy* / Q000378:metabolism*; D006801:Humans; D007700:Kinetics; D007930:Leucine / Q000378:metabolism; D008297:Male; D008875:Middle Aged
D007930:Leucine; D005947:Glucose; D000409:Alanine