ISSN:
1432-1440
Keywords:
AMP deaminase
;
Myoadenylate deaminase deficiency
;
Muscular exercise
;
Hypoxanthine
;
Ammonia
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Summary Three patients with primary myoadenylate deaminase deficiency were subjected to exercise on a bicycle ergometer at 125 W for 30 minutes. Blood samples prior to, during, and at the end of exercise were analyzed for lactate, ammonia, and hypoxanthine. In addition, urinary hypoxanthine excretion was measured. In these patients the serum lactate level increased to concentrations between 7.9 and 9.0 mmol/1 at the end of exercise whereas the mean lactate level in nine control subjects at the end of exercise was 3.3 mmol/l (range 1.1–8.1 mmol/l). There was no difference to control subjects in the exercise-induced increase in plasma levels of ammonia and hypoxanthine or in the increase in urinary hypoxanthine excretion. The findings support the hypothesis of a reduced substrate supply to the citric acid cycle in myoadenylate deaminase deficiency. The normal formation of ammonia and hypoxanthine excludes a marked loss of adenine nucleotides in working muscles in these patients.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00180060
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