Increased ammonia production during forearm ischemic work test in McArdle's disease.
A patient with typical features of late onset McArdle's disease is described. During forearm ischemic work test the patient exhibited an exaggerated increase in ammonia release, largely exceeding normal values. It is suggested, that this is due to an activation of the myokinase/myoadenylate deaminase pathway. Besides lack of lactate release increased ammonia release during ischemia may be a typical feature of McArdle's disease.
D000641:Ammonia / Q000378:metabolism*; D005542:Forearm / Q000098:blood supply*; D006003:Glycogen / Q000378:metabolism; D006008:Glycogen Storage Disease / Q000378:metabolism*; D006012:Glycogen Storage Disease Type V / Q000378:metabolism*; D006801:Humans; D007511:Ischemia / Q000378:metabolism*; D007773:Lactates / Q000378:metabolism; D019344:Lactic Acid; D008297:Male; D008875:Middle Aged; D005082:Physical Exertion
D007773:Lactates; D019344:Lactic Acid; D000641:Ammonia; D006003:Glycogen