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  • 1
    Electronic Resource
    Electronic Resource
    Competitive action of divalent cations and D600 in frog slow muscle fibers (1989)
    Springer
    The journal of membrane biology 112 (1989), S. 185-192 
    Details
    ISSN: 1432-1424
    Keywords: slow fiber ; K contracture ; D600 ; divalent cations ; competitive binding
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Chemistry and Pharmacology
    Notes: Summary Single, slow muscle fibers fromRana temporaria were equilibrated in normal Ringer's. 95 mmol/liter K1-solution containing various concentrations of Ca2+, Ni2+, Mn2 or Mg2+ was applied, and the ensuing contractures were recorded isometrically. While peak tension (F max) was little affected, maintained tension (measured 1 min after onset of contracture) strongly depended on the concentration and species of divalent cations. Tension was maintained at its peak value in the presence of all species of divalent cations provided their concentrations were adequately increased. Dose-response curves were hyperbolic: Lineweaver-Burk plots revealed straight lines with different slopes intersecting near 1/F max, and indicating the following order of efficiency: Ni2+〉Ca2+〉Mn2+〉〉Mg2+. Hill plots for these cations resulted in straight lines with slopes near 1. Qualitatively similar relationships were obtained with contracture solutions containing D6000 (3–12 μmol/liter). However, under these conditions higher concentrations of Ca2+ or Ni2+ were required in order to fully maintain tension. After a step concentration change in the medium during contracture, the effects of Ca2+ or D600 were detectable only after a delay of 9 and 18 sec, respectively. It is concluded that divalent cations and D600 compete for the same binding site according to a 1:1 reaction. This site is presumably located inside the transverse tubular system and controls inactivation of the contractile force.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01871279
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  • 2
    Electronic Resource
    Electronic Resource
    Long-term development of intelligence (IQ) and EEG in 34 children with phenylketonuria treated early (1988)
    Springer
    European journal of pediatrics 147 (1988), S. 361-367 
    Details
    ISSN: 1432-1076
    Keywords: CNS development ; Dietary treatment ; EEG ; IQ ; Phenylketonuria
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In 34 children with phenylketonuria (PKU) treated early the prognostic value of the age on institution of the diet (within the first 3 months of life) and of the quality of dietary treatment was determined in two different ways: 1) following intelligence closely (IQ) and (2) evaluating the EEG development up to their 12th (n=34) and 15th (n=18) years of life as appropriate. In general, IQ scores were found to be normal from the 4th–15th years of life. In our group of patients there was no effect on the IQ of the timing of diet onset. Children with “strict” dietary control showed a significantly higher IQ than those with “loose” control. One hundred and fifty-four EEGs (10/20 system, awake with eyes closed) were recorded at intervals of 2 years and conventionally evaluated. The development of alpha-activity was found to be normal. Beta-activity was enhanced. Abnormal EEG findings like general slowing and generalized paroxysmal activity (GPA) with or without spikes were more frequent in children with PKU than in controls, with the exception of focal abnormalities. EEG abnormalities increased with advancing age independently of IQ development and showed no relation to either the age at the onset nor the quality of dietary treatment.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00496411
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  • 3
    Electronic Resource
    Electronic Resource
    Prenatal diagnosis of “dihydrobiopterin synthetase” deficiency, a variant form of phenylketonuria (1986)
    Springer
    European journal of pediatrics 145 (1986), S. 176-178 
    Details
    ISSN: 1432-1076
    Keywords: Phenylketonuria ; Biopterin ; Phosphate-eliminating enzyme ; 6-Pyruvoyl tetrahydropterin synthase ; Amniotic fluid ; Erythrocytes
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Amniocentesis was performed at 19 weeks gestation in a mother who had previously delivered a boy with “dihydrobiopterin synthetase” (DHBS) deficiency. The amniotic fluid contained neopterin in high (136 nmol/l) and biopterin in very low concentrations (1.8 nmol/l). The activity of the phosphate-eliminating enzyme (PEE, also called 6-pyruvoyl tetrahydropterin synthase, substrate: 7,8-dihydroneoptein triphosphate) which is present in liver and erythrocytes and defective in DHBS deficiency, was measured in the erythrocytes of the family members. The fetal sample showed only 2% of the activity of healthy adult controls and was comparable with that of the affected sibling. Obligate heterozygotes had activities around 20% of the controls. Two fetal control samples showed even higher activities than adult erythrocytes. Sepiapterin reductase activities were normal in all cases. At autopsy, PEE deficiency was confirmed in the liver of the fetus. We concluded that DHBS deficiency (and most probably also GTP cyclohydrolase I deficiency) can be diagnosed by metabolite measurements in amniotic fluid. PEE activity is measurable in erythrocytes, although the assay needs to be improved. Since maternal tetrahydrobiopterin does not cross the placenta, treatment of a tetrahydrobiopterin-deficient fetus with tetrahydrobiopterin in utero is not possible.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00446058
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  • 4
    Electronic Resource
    Electronic Resource
    Continuation vs discontinuation of low-phenylalanine diet in PKU adolescents (1987)
    Springer
    European journal of pediatrics 146 (1987), S. A17 
    Details
    ISSN: 1432-1076
    Keywords: Phenylketonuria ; Adolescents ; Dietary control ; Intelligence ; Psychological problems
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00442050
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  • 5
    Electronic Resource
    Electronic Resource
    Neurological outcome in adult patients with early-treated phenylketonuria (1998)
    Springer
    European journal of pediatrics 157 (1998), S. 824-830 
    Details
    ISSN: 1432-1076
    Keywords: Key words Intelligence ; Neurology ; Neuropsychology ; Phenylketonuria
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Due to the observation of severe neurological symptoms in single patients as well as brain imaging, neuropsychological and neurophysiological abnormalities, the long-term prognosis of treated phenylketonuria is still under discussion. We investigated the neurological outcome of 57 (24 male, 33 female) patients with phenylketonuria (diet onset 〈3 months) at a mean age of 23.6 (17–33) years in comparison to control subjects. Methods used were a clinical-neurological examination, tests for fine motor abilities, IQ test (WAIS-R), a neuropsychological attention task and MRI (30 patients only). Tremor was increased in the patients (28%) compared to controls (15%). Fine motor abilities were significantly reduced in three areas: hand-wrist steadiness, finger-hand dexterity and hand-wrist speed. Tremor as well as reduced fine motor skills were not associated with treatment-related variables, e.g. diet onset, strictness of biochemical control or amount of MRI white matter change. IQ was lower in patients (mean 97.6) compared to matched control subjects (mean 105.5). IQ at 12 years was correlated with biochemical control from birth up to the age of 12 and remained stable up to adult age, independent of biochemical control after 12 years of age. In contrast to the other outcome parameters, the performance in a neuropsychological attention task was influenced by the concurrent plasma phenylalanine concentration. Specific late-onset neurological impairment was not identified in this sample of early-treated adults with phenylketonuria. Conclusion Careful neurological investigation revealed subtle symptoms of brain damage even after early-initiated treatment in adult patients with phenylketonuria. At present it cannot be excluded that further neurological deterioration could emerge later in life. Thus, patients with phenylketonuria – either on or off diet – should be monitored throughout life.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004310050945
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  • 6
    Electronic Resource
    Electronic Resource
    D600 prolongs inactivation of the contractile system in frog twitch fibres (1987)
    Springer
    Pflügers Archiv 410 (1987), S. 75-82 
    Details
    ISSN: 1432-2013
    Keywords: Twitch fibre ; K-contracture ; Inactivation ; D600
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract (1) Single twitch fibres were dissected from tibialis muscles ofRana temporaria and used to study the effect of D600 (gallopamil) on potassium-induced contractures. (2) 95 mM K-Ringer's was applied for 8–15 s at intervals of generally 2.5–5 min; at temperatures of 6–8°C and in the absence of D600 the amplitude of the contractures remained fairly constant. After pretreatment with D600 (30 μM) a single (conditioning) K-contracture was sufficient to ‘paralyze’ the fibres (cf. Eisenberg et al. 1983). (3) Complete paralysis could also be achieved at 18–20°C. In three fibres a single conditioning K-application was sufficient; in two more fibres two or three conditioning K-applications were required. (4) D600-paralysis could not only be achieved with high K-concentrations but also by conditioning with subor suprathreshold K-concentrations (20–40 mM); the duration of the conditioning periods required to induce complete paralysis was approximately the same before and after D600-treatment. (5) Contractures were partially abolished by application of 20–40 mM K-Ringer's for short conditioning periods; after D600-treatment the degree of contracture loss was similar. (6) At low temperature the state of partial or complete paralysis induced by subthreshold K-concentrations and D600 was maintained for long periods of time. (7) The presence of 10 mM Ca2+ did not protect the fibres from being paralyzed by treatment with D600 and high K-Ringer's at low temperature; however, more than one conditioning K-application was required. (8) Resting and action potentials of paralyzed fibres were not significantly different from control values. However, endplate potentials were reduced in size, and failure of neuromuscular transmission was observed in some of these fibres. (9) It is concluded that D600 prolongs the state of inactivation of the contractile mechanism, while the development of inactivation does not seem to be markedly affected.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00581899
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  • 7
    Electronic Resource
    Electronic Resource
    The effect of D600 on potassium contractures of slow muscle fibres ofRana temporaria (1988)
    Springer
    Pflügers Archiv 412 (1988), S. 390-396 
    Details
    ISSN: 1432-2013
    Keywords: Slow fibre ; K-Contracture ; D600 ; Inactivation ; Repriming
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract (1) The effect of 30 μM D600 on the amplitude and time course of isometric contractures was studied in single slow fibres ofRana temporaria. (2) D600 only slightly reduced the amplitude of contractures evoked with 30 or 95 mM K-Ringer's. Maintenance of tension was strongly impaired by D600 only during exposure to 95 mM K. The caffeine contracture was not affected. (3) Addition of 10 mM Ca2+ or other divalent cations to the medium strongly counteracted the effect of D600 on maintained tension. The order of efficiency was Ca2+=Ni2+〉Co2+〉Mn2+〉Mg2+. (4) During 2 min exposure to 95 mM K-Ringer's the slow fibres inactivated to a variable degree; recovery from inactivation in normal Ringer's proceeded with a half time of the order of 1 min, while in the presence of D600 recovery was prolonged 3.3 to 27 times. (5) It is concluded that the effect of D600 on the contractile behaviour of slow fibres fromRana temporaria is predominantly due to a prolongation of the inactivated state. It is suggested that D600 binds to a site at the outer membrane surface which also binds divalent cations and determines the degree of contractile inactivation during exposure to potassium. Blocking of Ca2+ channels is unlikely to be the mechanism of this D600-effect.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01907557
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