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  • 1
    Electronic Resource
    Electronic Resource
    Activity of orotate metabolizing enzyme complex and various urea-cycle enzymes in mutant mice with ornithine transcarbamylase deficiency (1982)
    Springer
    Cellular and molecular life sciences 38 (1982), S. 308-309 
    Details
    ISSN: 1420-9071
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary The overall activity of the enzyme complex consisting of orotate phosphoribosyl transferase and orotidine monophosphate decarboxylase, and of various enzymes of the urea cycle, has been studied in sparse-fur (spf) mutant mice with ornithine transcarbamylase deficiency. The enzyme complex has a lower overall activity, which could be caused by disturbed pyrimidine metabolism due to hyperammonemia. Other enzymes of the urea cycle do not show any significant change.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01949358
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Acquired carnitine abnormalities in critically ill children (1997)
    Springer
    European journal of pediatrics 156 (1997), S. 864-869 
    Details
    ISSN: 1432-1076
    Keywords: Key words Carnitine ; Child ; Head injury ; Heart defects ; Sepsis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In order to characterize the role of carnitine during metabolic stress, we prospectively determined carnitine profiles in plasma and urine on admission, days 2, 5, 10 and 15, among 28 critically ill children free of any known conditions associated with secondary carnitine deficiency. More than 25% of plasma and 50% of urinary carnitine measurements were abnormal; 96% (27/28) of patients displayed on at least one occasion an abnormal [〈−2 SD or 〉+2 SD] carnitine value in plasma. Three children had extremely low [〈10 μmol/l] free carnitine (FC) levels in plasma. Plasma esterified and FC levels on admission were not related to the risk of mortality [PRISM score], to muscle lysis [CK values], and to the caloric intake. Levels of FC and esterified carnitine in plasma were unrelated to those measured in urine. Conclusion Abnormal plasma and urine carnitine measurements are frequently found in critically ill children; the biological significance of these perturbations remains unclear. Caution must be exercised before concluding that an abnormal carnitine value is indicative of an underlying hereditary metabolic disorder in this population.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004310050732
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  • 3
    Electronic Resource
    Electronic Resource
    The ocular hypotensive effect of late pregnancy is higher in multigravidae than in primigravidae (2000)
    Springer
    Graefe's archive for clinical and experimental ophthalmology 238 (2000), S. 64-67 
    Details
    ISSN: 1435-702X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  · Background: Systemic hypertension and degenerative vascular disease are more common in multigravidae than in primigravidae. The present study investigated whether the known ocular hypotensive effect of late pregnancy is influenced by the number of pregnancies. · Methods: Intraocular pressure (IOP) was measured in normotensive third-trimester primigravidae and multigravidae along with nulligravida controls by means of the Goldmann applanation tonometer. Depending upon the number of previous pregnancies, multigravidae were divided into four subgroups. · Results: The IOP of the pregnant group (primigravidae and multigravidae together) was (mean±SEM) 2.1±0.07 mmHg (P〈0.001) lower than in the nulligravida control group. The IOP of nulligravidae was 1.7±0.06 mmHg (P〈0.001) and 2.5±0.01 mmHg (P〈0.001) higher than in third-trimester primigravidae and multigravidae, respectively. In all subgroups of multigravidae IOP was significantly lower (P〈0.02) than in primigravidae. The differences among different subgroups of multigravidae were statistically insignificant. · Conclusions: Gravidity influences IOP and should be taken into account in future research.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004170050011
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  • 4
    Electronic Resource
    Electronic Resource
    A new French-Canadian family affected by hyperargininaemia (1983)
    Springer
    Journal of inherited metabolic disease 6 (1983), S. 179-182 
    Details
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A new French-Canadian family from the province of Quebec is reported, in which a male child was diagnosed as hyperargininaemic after showing positive tests for cystimuria on neonatal screening. The child has no residual activity of erythrocyte arginase, and a plasma arginine level of 633 μmol/l. Both parents have 32–38% of arginase activity. A newborn sister has normal enzyme levels. The propositius did not show abnormal plasma ammonia elevation even after a protein tolerance test (1.5 g protein/kg body weight) but excretes high levels of urinary orotate (845 mg/g creatinine). At 3 1/2 years of age the hyperargininaemic child had started showing abnormal gait, ataxia and slowing of intellectual development. It is suggested that all newborn children showing cystimuria-lysinuria pattern of amino acid excretion be tested for arginase deficiency.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02310878
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  • 5
    Electronic Resource
    Electronic Resource
    Kinetic abnormalities of carbamyl phosphate synthetase-I in a case of congenital hyperammonaemia (1986)
    Springer
    Journal of inherited metabolic disease 9 (1986), S. 253-260 
    Details
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A sensitive direct colourimetric method has been employed to measure kinetic parameters and pH dependence of carbamyl phosphate synthetase-I, in a liver sample from a 2 1/2-month-old girl, who died from complications of a late-developing congenital hyperammonaemia. The residual activity of carbamyl phosphate synthetase-I was 25%, whereas other urea cycle enzymes were within normal range. ApparentK m for ammonium ion (0.73 mmol/L) was significantly increased (normal range 0.24–0.51).K m for bicarbonate ion was normal, whileK m for NAG showed a slight variation from normal. The pH dependence curve of the patient's enzyme was flat, as compared to two controls showing pH optima at 7.8. Radial immunodiffusion (Mancini) of the abnormal enzyme against human enzyme antiserum gave a cross-reacting material of 10–20%. The methodological approach presented can be used to characterize abnormal enzymes in cases of partial deficiency with only 100–200 mg of liver tissue.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01799657
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    Paper (German National Licenses)
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