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

David MacLean, John Vissing, Susanne F. Vissing, Ronald G. Haller

Research output: Contribution to journalArticle

31 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)1456-1459
Number of pages4
JournalNeurology
Volume51
Issue number5
DOIs
StatePublished - Nov 1998

ASJC Scopus subject areas

  • Clinical Neurology

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