Oral branched-chain amino acids do not improve exercise capacity in McArdle disease

Neurology

Volumn 51, Issue 5, 1998, Pages 1456-1459

  MacLean, David ^a   Vissing, John ^a   Vissing, Susanne F ^a   Haller, Ronald G ^b,c,d  

^a University of Copenhagen   (Denmark)

^b VA NORTH TEXAS HEALTH CARE SYSTEM   (United States)

^c UNIVERSITY OF TEXAS AT DALLAS   (United States)

^d IEEM   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ALANINE; BRANCHED CHAIN AMINO ACID; FATTY ACID; GLUTAMINE; GLYCOGEN PHOSPHORYLASE; ISOLEUCINE; LEUCINE; VALINE;

ADULT; AMINO ACID METABOLISM; ARTICLE; CLINICAL ARTICLE; CONTROLLED STUDY; EXERCISE; FEMALE; GLYCOGEN STORAGE DISEASE TYPE 5; HUMAN; MALE; PRIORITY JOURNAL;

EID: 0031743795     PISSN: 00283878     EISSN: None     Source Type: Journal    
DOI: 10.1212/WNL.51.5.1456     Document Type: Article

References (10)

1. Lewis SF, Haller RG. Human disorders of muscle glycogenolysis/glycolysis: the consequences of substrate-limited oxidative metabolism. In: Taylor AW, ed. Biochemistry of exercise VII. Champaign, IL: Human Kinetics, 1990:211-226.
2. Tawa NEJ, Goldberg AL. Protein and amino acid metabolism in muscle. In: Engel AG, Franzini-Armstrong C, eds. Myology: basic and clinical. New York: McGraw-Hill, 1994:683-707.
3. Wahren J, Felig P, Havel R, Jorfeldt L, Pernow B, Saltin B. Amino acid metabolism in McArdle's syndrome. N Engl J Med 1973;288:774-777.
4. Slonim A, Goans P. Myopathy in McArdle's syndrome. Improvement with a high-protein diet. N Engl J Med 1985;312: 355-359.
5. Jensen KE, Jakobsen J, Thomsen C, Henriksen O. Improved energy kinetics following high protein diet in McArdle's syndrome. A 31P magnetic resonance spectroscopy study. Acta Neurol Scand 1990;81:499-503.
6. Coakley JH, Wagenmakers AJ, Edwards RH. Relationship between ammonia, heart rate, and exertion in McArdle's disease. Am J Physiol 1992;262:E167-E172.
7. Vissing J, Lewis SF, Galbo H, Haller RG. Effect of deficient muscular glycogenolysis on extramuscular fuel production in exercise. J Appl Physiol 1992;72:1773-1779.
8. MacLean DA, Graham TE, Saltin B. Stimulation of muscle ammonia production during exercise following branched-chain amino acid supplementation in humans. J Physiol 1996;493: 909-922.
9. Wagenmakers AJ, Coakley JH, Edwards RH. Metabolism of branched-chain amino acids and ammonia during exercise: clues from McArdle's disease. Int J Sports Med 1990;11:S101-S113.
10. Pernow BB, Havel RJ, Jennings DB. The second wind phenomenon in McArdle's syndrome. Acta Med Scand 1967; 472(suppl):294-307.