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  • Chemistry  (91)
  • Polymer and Materials Science  (23)
  • Phenylketonuria  (4)
  • Scorpion Venom  (3)
  • 1
    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.
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  • 2
    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.
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 157 (1998), S. 824-830 
    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.
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 146 (1987), S. A17 
    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
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Pflügers Archiv 303 (1968), S. 133-149 
    ISSN: 1432-2013
    Keywords: Node of Ranvier ; Ionic Currents ; Scorpion Venom ; Ranvierscher Schnürring ; Ionenströme ; Skorpiongift
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: 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.
    Notes: 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.
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Pflügers Archiv 303 (1968), S. 150-161 
    ISSN: 1432-2013
    Keywords: Node of Ranvier ; Sodium Inactivation ; Scorpion Venom ; Ranvierscher Schnürring ; Natrium-Inaktivierung ; Skorpiongift
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: 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.
    Notes: 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.
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Pflügers Archiv 333 (1972), S. 51-61 
    ISSN: 1432-2013
    Keywords: Node of Ranvier ; Ionic Currents ; Scorpion Venom ; Calcium Ions
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: 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.
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  • 8
    Electronic Resource
    Electronic Resource
    Weinheim : Wiley-Blackwell
    Angewandte Makromolekulare Chemie 248 (1997), S. 139-151 
    ISSN: 0003-3146
    Keywords: Chemistry ; Polymer and Materials Science
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Chemistry and Pharmacology , Physics
    Description / Table of Contents: Ein neuartiges organisch-anorganisches Hybridmaterial wurde durch den Einbau des Minerals Böhmit mittels eines Sol-Gel-Prozesses in ein Kevlar-Nomex-Copolymeres hergestellt. Durch Reaktion eines Gemisches aus m- und p-Phenylendiamin mit Terephthaloylchlorid und anschließender Umsetzung mit Aminophenyltrimethoxysilan wurden Poly(phenylenterephthalamid)-Ketten mit Aminophenyltri-methoxysilan-Endgruppen synthetisiert. In dieser Polymermatrix wurde die Hydrolyse der Alkoxygruppen einer Lösung von Aluminium-sec-butanolat in Butanol durchgeführt. Dadurch wurde ein chemisch an die Aramid-Ketten gebundenes anorganisches Netzwerk aufgebaut. Aus den hergestellten Hybridmaterialien mit unterschiedlichen Böhmit-Gehalten in der Aramidmatrix wurden Filme gegossen. Die Filme aus Hybridmaterial mit bis zu 15 Gew.-% Böhmit waren transparent gelb, während bei 20 und mehr Gew.-% Böhmit opake Filme erhalten wurden. Die mechanischen Eigenschaften dieser Filme wurden untersucht. Zugfestigkeit, Zugmodul, Härte und maximale Bruchdehnung nahmen mit steigendem Böhmitgehalt zunächst zu, vemngerten sich jedoch bei weiterer Erhöhung des Mineralanteils wieder. Der Zugmodul des Hybridmaterials bei 25°C lag zwischen 3,0 und 4,5 GPa. Die Materialien hielten einer maximalen Zugspannung von 252 MPa stand und zeigten Zersetzungstemperaturen von etwa 450°C. Die rnit DMTA ermittelten Werte für den Speichemodul lagen im Bereich 7,6-18,9 GPa. Das tan δ-Signal verbreiterte sich mit zunehmendem Böhmitanteil und ging bei hohen Böhmitgehalten in eine Schulter über. Die Signalpositionen des Verlustfaktors wurden infolge der zunehmenden sterischen Einschränkung der Segmentbewegungen rnit steigendem anorganischen Anteil zu höheren Temperaturen verschoben.
    Notes: A new organic-inorganic hybrid material was prepared by incorporating boehmite into a Kevlar-Nomex copolymer via a sol-gel process. Poly(phenylene-terephthalamide) chains having aminophenyltrimethoxysilane end groups were prepared by reacting a mixture of m-and p-phenylenediamine with terephthaloyl chloride, followed by end-capping with aminophenyltrimethoxysilane. The hydrolysis of the alkoxy groups of an aluminium sec-butoxide solution in butanol was carried out in the polymer matrix, thus creating an inorganic network structure combined chemically with the aramid chains. Hybrid materials containing different proportions of boehmite in aramid were thus produced and films were cast by solvent evaporation technique. These films with up to 15 wt.-% of boehmite were yellow and transparent, whereas the films with 20 wt.-% or more contents of boehmite were opaque. Mechanical properties of these films were analyzed. The values of tensile strength, initial modulus, toughness and maximum strain at rupture were initially found to increase and then decrease with further addition of boehmite. The tensile modulus of the hybrid material was found to be in the range of 3.0-4.5 GPa at 25°C. These ceramers were found to withstand maximum tensile stress of the order of 252 MPa, and the thermal decomposition temperature was around 450°C. The storage modulus as measured using DMTA was in the range of 7.6-18.9 GPa. The tan δ peak which in general became broader with increasing boehmite content showed a shoulder for composites containing large amounts of boehmite. The position of the peaks shifted towards high temperature with the increase in the inorganic contents showing hindrance in motion with increasing boehmite contents.
    Additional Material: 8 Ill.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 0021-8383
    Keywords: Chemistry ; Organic Chemistry
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Chemistry and Pharmacology
    Notes: Deactivation Behaviour of Arenes and Heteroarenes. XXXVI. The Solvent and Temperature Dependence of Deactivation Behaviour of 9, 9′-Bis(acridizinium-yl) at High ConcentrationAt room temperature the geometrical structure of the fluoresceing singlet state of 9, 9′-bis(acridizinium-yl) is determined by the solvent. In solvents of the type A (water, methanol, acetonitril) a plain S1-state emits, in the case of the solvent type B (ethanol, propanol-(2)) the S1-state is twisted. Both excited states have different emission spectroscopical properties.At high concentration (c ≥ 10-4 mol l-1) in the case of the solvent type B a new emission band is observed, and the monomer emission is quenched. This new band is interpreted as a fluorescence from a molecular associate in the excited state. The intensity of this associate band is increasing with decreasing temperature. It is shown that this band is the result of molecular association in the ground state. From the photochemical irradiation it follows that only in the case of the solvent type B a photocycloaddition is possible. The plain S1-state does not react.
    Additional Material: 3 Ill.
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  • 10
    Electronic Resource
    Electronic Resource
    Hoboken, NJ : Wiley-Blackwell
    AIChE Journal 35 (1989), S. 507-510 
    ISSN: 0001-1541
    Keywords: Chemistry ; Chemical Engineering
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Chemistry and Pharmacology , Process Engineering, Biotechnology, Nutrition Technology
    Additional Material: 6 Ill.
    Type of Medium: Electronic Resource
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