Document Detail

Relationship between ammonia, heart rate, and exertion in McArdle's disease.
MedLine Citation:
PMID:  1539642     Owner:  NLM     Status:  MEDLINE    
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.
J H Coakley; A J Wagenmakers; R H Edwards
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  The American journal of physiology     Volume:  262     ISSN:  0002-9513     ISO Abbreviation:  Am. J. Physiol.     Publication Date:  1992 Feb 
Date Detail:
Created Date:  1992-03-31     Completed Date:  1992-03-31     Revised Date:  2008-11-21    
Medline Journal Info:
Nlm Unique ID:  0370511     Medline TA:  Am J Physiol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  E167-72     Citation Subset:  IM    
Department of Medicine, University of Liverpool, United Kingdom.
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MeSH Terms
Amino Acids, Branched-Chain / pharmacology*
Ammonia / blood*
Drug Combinations
Exercise Test
Glucose / pharmacology
Glycogen Storage Disease Type V / blood,  physiopathology*
Heart Rate*
Keto Acids / pharmacology
Leucine / pharmacology
Middle Aged
Osmolar Concentration
Physical Endurance / drug effects
Physical Exertion*
Valine / pharmacology
Reg. No./Substance:
0/Amino Acids, Branched-Chain; 0/Drug Combinations; 0/Keto Acids; 50-99-7/Glucose; 61-90-5/Leucine; 7004-03-7/Valine; 7664-41-7/Ammonia

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