{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,14]],"date-time":"2026-03-14T03:46:44Z","timestamp":1773460004718,"version":"3.50.1"},"reference-count":23,"publisher":"American Physiological Society","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American Journal of Physiology-Endocrinology and Metabolism"],"published-print":{"date-parts":[[1992,2,1]]},"abstract":"<jats:p> We studied plasma ammonia and exercise tolerance in six patients with McArdle's disease (myophosphorylase deficiency, type V glycogenosis) during incremental cycle ergometry. Tests were performed either in the postabsorptive state or after supplementation with branched-chain amino and 2-oxoacids and glucose. Glucose and branched-chain 2-oxoacid combined increased total work performed from control 49 +\/- 22 to 80 +\/- 36 kJ (P less than 0.05). Glucose alone also improved total work performed from 49 +\/- 22 to 64 +\/- 33 kJ (P less than 0.05). Branched-chain 2-oxoacids alone had a variable effect, and branched-chain amino acids were of no benefit. Correlations between plasma ammonia and heart rate for individual patients were r = 0.99, P less than 0.01; r = 0.95, P less than 0.01; r = 0.84, P less than 0.01; r = 0.76, P less than 0.01; r = 0.73, P less than 0.01; and r = 0.63, P less than 0.05 and between ammonia and perceived exertion for all patients combined was r = 0.70, P less than 0.0001. In two patients, correlation of ammonia with heart rate at a power output of 60 W was r = 0.91, P less than 0.001 and at 40 W was r = 0.77, P less than 0.001. We conclude that ammonia is either a mediator or a marker of the metabolic events leading to fatigue. <\/jats:p>","DOI":"10.1152\/ajpendo.1992.262.2.e167","type":"journal-article","created":{"date-parts":[[2017,12,21]],"date-time":"2017-12-21T23:09:33Z","timestamp":1513897773000},"page":"E167-E172","source":"Crossref","is-referenced-by-count":10,"title":["Relationship between ammonia, heart rate, and exertion in McArdle's disease"],"prefix":"10.1152","volume":"262","author":[{"given":"J. H.","family":"Coakley","sequence":"first","affiliation":[{"name":"Department of Medicine, University of Liverpool, United Kingdom."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A. J.","family":"Wagenmakers","sequence":"additional","affiliation":[{"name":"Department of Medicine, University of Liverpool, United Kingdom."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R. 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