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

From the Copenhagen Muscle Research Centre (Drs. MacLean, J. Vissing, and S.F. Vissing); the Department of Neurology (Dr. J. Vissing), Rigshospitalet, Copenhagen, Denmark; and the Neuromuscular Center (Dr. Haller), Institute for Exercise and Environmental Medicine of Presbyterian Hospital, Dallas VA Medical Center, and Department of Neurology, University of Texas Southwestern Medical Center, Dallas TX.

Address correspondence and reprint requests to Dr. Ronald G. Haller, Neuromuscular Center, IEEM, 7232 Greenville Avenue, Suite 435, Dallas, TX 75231.

To determine whether oral branched-chain amino acids (BCAAs) improve exercise capacity, six fasting patients with McArdle's disease were given a solution of BCAA (77 mg/kg) or a control noncaloric beverage 30 minutes before exercise. The BCAA meal tripled plasma BCAA levels, increased BCAA catabolism as indicated by greater exercise increases in plasma glutamine and alanine, but lowered mean peak free fatty acid levels and reduced exercise capacity in five of six patients. Lower work capacity may be attributed to a net reduction in muscle fuel availability after BCAA administration.

Supported by the Danish National Research Foundation (504-14), a Veterans Administration Merit Review, and by grants from the National Aeronautics and Space Administration and the Muscular Dystrophy Association.

Received December 12, 1997. Accepted in final form July 18, 1998.

This article has been cited by other articles:

				S. T. Andersen, R. G. Haller, and J. Vissing Effect of Oral Sucrose Shortly Before Exercise on Work Capacity in McArdle Disease Arch Neurol, June 1, 2008; 65(6): 786 - 789. [Abstract] [Full Text] [PDF]

				J. Vissing and R. G. Haller The Effect of Oral Sucrose on Exercise Tolerance in Patients with McArdle's Disease N. Engl. J. Med., December 25, 2003; 349(26): 2503 - 2509. [Abstract] [Full Text] [PDF]

				R. G. Haller Treatment of McArdle Disease Arch Neurol, July 1, 2000; 57(7): 923 - 924. [Full Text] [PDF]