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  • Artikel: DFG Deutsche Nationallizenzen  (8)
  • Node of Ranvier  (4)
  • Phenylketonuria  (4)
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  • Artikel: DFG Deutsche Nationallizenzen  (8)
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  • 1
    Digitale Medien
    Digitale Medien
    Effect of aconitine on the sodium permeability of the node of ranvier (1974)
    Springer
    Pflügers Archiv 349 (1974), S. 133-148 
    Details
    ISSN: 1432-2013
    Schlagwort(e): Node of Ranvier ; Sodium Permeability ; Aconitine
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Summary The effect of aconitine (10−5–10−6 g/ml) on membrane potentials and membrane currents of myelinated nerve fibres of Xenopus laevis was investigated. The following observations were made: a) Current clamp conditions: Slow depolarization (10–15 mV), decrease of amplitude and maximum rate of rise of action potential, finally inexcitability. With inward current pulses ‘hyperpolarizing responses’ could be elicited at membrane potentials more negative than the resting potential (E r ). Neither spontaneous activity nor repetitive responses to electrical stimuli were observed. No effects of aconitine were found in Na-free solutions or in the presence of tetrodotoxin. b) Voltage clamp conditions: Development of steady inward current at normal resting potential due to formation of a non-inactivating sodium permeability; heavily poisoned nodes therefore exhibit an N-shaped steady-state current voltage relation with negative slope at membrane potentials more negative thanE r . These non-inactivating sodium channels open more slowly than normal sodium channels, and can only be closed by hyperpolarizing the membrane by about 50 mV. The majority of sodium channels have almost normalτ m; theirm ∞ andh ∞-V relations are shifted by 10–15 mV towardsE r . It is concluded that these changes of the sodium permeability account for the changes of electrical activity observed after treatment with aconitine.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00586624
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  • 2
    Digitale Medien
    Digitale Medien
    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
    Schlagwort(e): CNS development ; Dietary treatment ; EEG ; IQ ; Phenylketonuria
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: 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.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00496411
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  • 3
    Digitale Medien
    Digitale Medien
    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
    Schlagwort(e): Phenylketonuria ; Biopterin ; Phosphate-eliminating enzyme ; 6-Pyruvoyl tetrahydropterin synthase ; Amniotic fluid ; Erythrocytes
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: 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.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00446058
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  • 4
    Digitale Medien
    Digitale Medien
    Neurological outcome in adult patients with early-treated phenylketonuria (1998)
    Springer
    European journal of pediatrics 157 (1998), S. 824-830 
    Details
    ISSN: 1432-1076
    Schlagwort(e): Key words Intelligence ; Neurology ; Neuropsychology ; Phenylketonuria
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: 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.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/s004310050945
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  • 5
    Digitale Medien
    Digitale Medien
    Continuation vs discontinuation of low-phenylalanine diet in PKU adolescents (1987)
    Springer
    European journal of pediatrics 146 (1987), S. A17 
    Details
    ISSN: 1432-1076
    Schlagwort(e): Phenylketonuria ; Adolescents ; Dietary control ; Intelligence ; Psychological problems
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00442050
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  • 6
    Digitale Medien
    Digitale Medien
    Die Wirkung von Skorpiongift auf die Ionenströme des Ranvierschen Schnürrings (1968)
    Springer
    Pflügers Archiv 303 (1968), S. 133-149 
    Details
    ISSN: 1432-2013
    Schlagwort(e): Node of Ranvier ; Ionic Currents ; Scorpion Venom ; Ranvierscher Schnürring ; Ionenströme ; Skorpiongift
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Beschreibung / Inhaltsverzeichnis: Zusammenfassung 1. Die Wirkung von Skorpiongift (10−6 g/ml) auf die Ionenströme einzelner markhaltiger Nervenfasern von Xenopus laevis wurde in voltage-clamp-Experimenten untersucht. 2. Skorpiongift beeinflußt sowohl die Natrium- als auch die Kalium-Permeabilität der Schnürringmembran. 3. Die hauptsächlichen Wirkungen des Skorpiongiftes auf die Natrium-PermeabilitätP Na sind: a) Die maximale Natrium-Permeabilität $$\bar P_{_{{\rm N}a} } $$ wird auf 60% ihres an der unvergifteten Membran gemessenen Wertes reduziert. b) Die Natriuminaktivierung wird stark verlangsamt und verläuft in zwei Phasen. c) Der zeitliche Verlauf vonP Na kann unter der Annahme beschrieben werden, daß die Variableh aus zwei Komponentenx undy besteht, deren Zeitkonstanten τ x und τ y 4 bzw. 200 mal so groß sind wie τ h der unvergifteten Membran. 4. Die Wirkungen des Skorpiongiftes auf die Kalium-PermeabilitätP K sind: a) Die maximale Kalium-PermeabilitätP K' wird auf etwa 35% ihres Normalwertes vermindert. b) Der Anstieg vonP K bei Depolarisation wird verlangsamt; zwei Phasen können unterschieden werden. 5. Die Wirkungen auf die Natrium-Inaktivierung und auf die Kalium-Permeabilität können die unter dem Einfluß von Skorpiongift beobachtete extreme Verlängerung des Aktionspotentials erklären.
    Notizen: Summary 1. The action of scorpion venom (10−6 g/ml) on the ionic currents of single myelinated nerve fibres of Xenopus laevis was studied in voltage clamp experiments. 2. Scorpion venom was found to affect the sodium permeability as well as the potassium permeability of the nodal membrane. 3. The main effects of scorpion venom on the sodium permeabilityP Na are: a) The maximum sodium permeability $$\bar P_{_{{\rm N}a} } $$ is reduced to 60% of its value in the unpoisoned membrane. b) The sodium inactivation is markedly slowed and proceeds in two phases. c) The time course ofP Na can be described assuming that the variableh is the sum of two componentsx andy, whose time constants τ x and τ y are 4 and 200 times τh of the unpoisoned membrane. 4. The effects of scorpion venom on the potassium permeabilityP K are: a) The maximum potassium permeabilityP K' is reduced to about 35% of its normal value. b) The increase ofP K' upon depolarisation is slowed down; two phases can be distinguished. 5. The effects on sodium inactivation and potassium permeability may explain the extreme prolongation of the action potential under the influence of scorpion venom.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00592631
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  • 7
    Digitale Medien
    Digitale Medien
    Die Wirkung von Skorpiongift auf die Ionenströme des Ranvierschen Schnürrings (1968)
    Springer
    Pflügers Archiv 303 (1968), S. 150-161 
    Details
    ISSN: 1432-2013
    Schlagwort(e): Node of Ranvier ; Sodium Inactivation ; Scorpion Venom ; Ranvierscher Schnürring ; Natrium-Inaktivierung ; Skorpiongift
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Beschreibung / Inhaltsverzeichnis: Zusammenfassung 1. Die Wirkung von Skorpiongift auf die Inaktivierung der Natrium-PermeabilitätP Na wurde in voltage-clamp-Experimenten an einzelnen markhaltigen Nervenfasern von Xenopus laevis untersucht. 2. Beim normalen Ruhepotential warP Na der vergifteten Membran stärker inaktiviert als an der unvergifteten Membran; bei starken Depolarisationen war die Inaktivierung jedoch unvollständig. 3. Der zeitliche Verlauf vonP Na konnte unter verschiedenen experimentellen Bedingungen mit den Hodgkin-Huxley-Gleichungen beschrieben werden, die wie folgt modifiziert wurden: a) die Variableh wurde durch eine schnelle Komponentex und eine langsame Komponentey ersetzt; b) die Komponentex nahm selbst bei Depolarisationen von mehr als 40 mV nicht auf Null ab.
    Notizen: Summary 1. The action of scorpion venom on the inactivation of the sodium permeabilityP Na was studied in voltage-clamp experiments on single myelinated nerve fibres of Xenopus laevis. 2. At the normal resting potentialP Na of the poisoned membrane was more inactivated than the control; at large depolarizations inactivation was, however, incomplete. 3. The time course ofP Na under various experimental conditions could be described by theHodgkin-Huxley equations modified as follows: a) the variableh was split into a fast and a slow component (x andy, respectively); b) componentx did not vanish even for depolarizations by more than 40 mV.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00592632
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  • 8
    Digitale Medien
    Digitale Medien
    Influence of calcium ions on the ionic currents of nodes of Ranvier treated with scorpion venom (1972)
    Springer
    Pflügers Archiv 333 (1972), S. 51-61 
    Details
    ISSN: 1432-2013
    Schlagwort(e): Node of Ranvier ; Ionic Currents ; Scorpion Venom ; Calcium Ions
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Summary Voltage clamp experiments were done on myelinated nerve fibres of Xenopus laevis to study the effect of calcium on the ionic currents of the nodal membrane treated with scorpion venom. Increasing the calcium concentration of the medium from 1.8 to 7.2 mM produced the following changes of the sodium and potassium permeabilities,P Na andP K: a) The incomplete sodium inactivation, which is typical for nodes of Ranvier treated with scorpion venom, is abolished. The curves relatingm ∞ and τ m to membrane potential are shifted by 8–10 mV towards larger depolarizations.P Na and the time constants of the fast and slow components of the sodium inactivation remain practically unchanged. b) The maximum potassium permeability,P K, which is reduced by scorpion venom to 45% of the value observed in the normal node, increases to 68% by addition of calcium ions; τ n is unchanged. These effects of calcium ions on the ionic permeabilities explain the shortening of the extremely prolonged action potential and the inhibition of spontaneous activity observed in nodes of Ranvier poisoned with scorpion venom.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00586041
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