Library

X
feed icon rss

Your email was sent successfully. Check your inbox.

An error occurred while sending the email. Please try again.

Proceed reservation?

Export
  • 1
    Electronic Resource
    Electronic Resource
    Ribose administration during exercise: Effects on substrates and products of energy metabolism in healthy subjects and a patient with myoadenylate deaminase deficiency (1991)
    Springer
    Journal of molecular medicine 69 (1991), S. 151-155 
    Details
    ISSN: 1432-1440
    Keywords: Myoadenylate deaminase ; AMP-deaminase ; Deficiency ; Ribose ; Lactate ; Ammonia ; Hypoxanthine ; Muscle ; Metabolism
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Nine healthy men and a patient with myoadenylate deaminase deficiency were exercised on a bicycle ergometer (30 minutes, 125 Watts) with and without oral ribose administration at a dose of 2 g every 5 minutes of exercise. Plasma or serum levels of glucose, free fatty acids, lactate, ammonia and hypoxanthine and the urinary hypoxanthine excretion were determined. After 30 minutes of exercise without ribose intake the healthy subjects showed significant increases in plasma lactate (p〈0.05), ammonia (p〈0.01) and hypoxanthine (p〈0.05) concentrations and a decrease in serum glucose concentration (p〈0.05). When ribose was administered, the plasma lactate concentration increased significantly higher (p〈 0.05) and the increase in plasma hypoxanthine concentration was no longer significant. The patient showed the same pattern of changes in serum or plasma concentrations with exercise with the exception of hypoxanthine in plasma which increased higher when ribose was administered.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01665856
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
  • 2
    Electronic Resource
    Electronic Resource
    Metabolism of D-ribose administered continuously to healthy persons and to patients with myoadenylate deaminase deficiency (1989)
    Springer
    Journal of molecular medicine 67 (1989), S. 1205-1213 
    Details
    ISSN: 1432-1440
    Keywords: (D-)ribose ; Metabolism ; Myoadenylate ; (adenylate-, AMP-)deaminase deficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary D-ribose was administered orally or intravenously over at least 5 h to eight healthy volunteers and five patients with myoadenylate deaminase deficiency. Intravenous administration rates were 83, 167, and 222 mg/kg/h, which were well tolerated but oral administration of more than 200 mg/kg/h caused diarrhea. The average steady state serum ribose level ranged between 4.8 mg/100 ml (83 mg/kg/h, oral administration) and 81.7 mg/100 ml (222 mg/kg/h, intravenous administration). Serum glucose level decreased during ribose administration. The intestinal absorption rate of orally administered ribose was 87.8%–99.8% of the intake at doses up to 200 mg/kg/h without first pass effect. Urinary losses were 23% of the intravenously administered dose at 222 mg/kg/h. Ribose appeared to be excreted by glomerular filtration without active reabsorption; a renal threshold could not be demonstrated. The amount of ribose transported back from the tubular lumen depended on the serum ribose level. There was no difference in ribose turnover in healthy subjects and patients with MAD deficiency.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01716208
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
  • 3
    Electronic Resource
    Electronic Resource
    Ergometer exercise in myoadenylate deaminase deficient patients (1993)
    Springer
    Journal of molecular medicine 71 (1993), S. 461-465 
    Details
    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
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
  • 4
    Electronic Resource
    Electronic Resource
    Myoadenylate deaminase deficiency with severe rhabdomyolysis (1993)
    Springer
    European journal of pediatrics 152 (1993), S. 513-515 
    Details
    ISSN: 1432-1076
    Keywords: Myoadenylate deaminase deficiency ; Rhabdomyolysis ; DNA-analysis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 13-year-old Turkish girl was admitted because of recurrent episodes of muscle pain and weakness since the age of 5 years. As an outpatient she developed severe acute rhabdomyolysis (myoglobinuria and increased serum creatine kinase level of 19000 units/l). The acute rhabdomyolysis and the preceding episodes of muscle pain and weakness had been induced by exercise. There was no increase in plasma ammonia level during ischaemic forearm exercise test and bicycle ergometry. Myoadenylate deaminase deficiency was proven both histochemically and biochemically. The girl was found to be homozygous for the C 34-T mutation of the AMPD1 gene causing primary myoadenylate deaminase deficiency in skeletal muscle. Both parents and her brother were heterozygous for that mutation. Myoadenylate deaminase deficiency has to be considered as a cause of severe rhabdomyolysis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01955062
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
Close ⊗
This website uses cookies and the analysis tool Matomo. More information can be found here...