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  • Polymer and Materials Science  (23)
  • Node of Ranvier  (4)
  • Key words Phenylketonuria  (3)
  • 1
    Electronic Resource
    Electronic Resource
    Intelligence and professional career in young adults treated early for phenylketonuria (1996)
    Springer
    European journal of pediatrics 155 (1996), S. S97 
    Details
    ISSN: 1432-1076
    Keywords: Key words Phenylketonuria ; Intelligence ; Education ; Professional career ; Phenylalanine ; control
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The intellectual status and professional careers of 51 young adults with phenylketonuria whose treatment started before 3 months of age are described. Their mean IQ was 97 (SD = 16). Of the IQs, 4% were more than 2 SD below the norm. The distribution of types of schooling of the patients was comparable to that in the German population. The professional careers of nearly all the patients were according to their educational level. Within the sample the outcome was significantly correlated with phenylalanine (Phe) control, even when the patients’ social background was statistically taken into account. The main influence of Phe on intelligence seems to occur during the first decade of life since IQ data remain stable even after Phe levels increased during adolescence.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/PL00014262
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  • 2
    Electronic Resource
    Electronic Resource
    Phenylalanine hydroxylase genotypes, predicted residual enzyme activity and phenotypic parameters of diagnosis and treatment of phenylketonuria (1996)
    Springer
    European journal of pediatrics 155 (1996), S. S11 
    Details
    ISSN: 1432-1076
    Keywords: Key words Phenylketonuria ; Genotype ; Treatment ; IQ
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The interdependence of the predicted in vitro residual enzyme activity (PRA), as deduced from the complete genotypes of 64 hyperphenylalaninaemic patients, and parameters for diagnosis of hyperphenylalaninaemic disorders, the fluctuation of the phyenlylalanine (Phe) values during treatment, long-term dietary control during treatment, and a parameter for the outcome of therapy (IQ) was investigated by correlation analysis. A highly significant correlation was found between the PRA and diagnostic parameters, as well as the fluctuation of the Phe values during treatment. Significant correlations were also observed between the parameter describing the fluctuation of the Phe values and the IQ, as well as between the quality of dietary control and IQ. The PRA is a valuable tool for the differential diagnosis of hyperphenylalaninaemic disorders and for the prediction of one aspect of the course of the disease which is related to the intellectual outcome of therapy. The quality of dietary control was independent of the genotype, indicating that the outcome of therapy can be successfully manipulated in spite of the genetic make-up.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/PL00014222
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  • 3
    Electronic Resource
    Electronic Resource
    Magnetic resonance imaging of the brain in adolescents with phenyketonuria and in one case of 6-pyruvoyl tetrahydropteridine synthase deficiency (1996)
    Springer
    European journal of pediatrics 155 (1996), S. S69 
    Details
    ISSN: 1432-1076
    Keywords: Key words Phenylketonuria ; Phenylalanine ; Magnetic resonance ; imaging ; Evoked potentials ; Myelination
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract White matter abnormalities on MRI have been observed in phenylketonuria (PKU) patients with late onset neurological symptoms as well as in neurologically inconspicious patients. We investigated 14 early treated adolescents at an age between 12 and 17 years (mean age 14.3 years) with classical PKU as well as one retarded patient with atypical PKU by cranial MRI with spinecho T1-, T2- and proton density sequences. Clinical examination was normal. Visual evoked potential (VEP) examination showed a prolonged latency of peak P100 (mean 122.6 ms; control mean 115.9) and IQ testing showed a mean IQ of 101.1. To investigate the influence of plasma phenylalanine (Phe) levels three approaches were used: Phe was determined for the day of MRI, for a period of 6 months prior to MRI and for lifetime up to 12 years. MRI scans revealed areas of abnormally increased signal intensity on T2-weighted and proton density images in 12 (86%) patients, preferably involving the parieto-occipital lobes. MRI of the patient with atypical PKU was normal. MRI findings correlated most strongly to long-term dietary control up to 12 years. We found no correlation with the other parameters of biochemical control, IQ or VEP latency. The nature and prognosis of MRI abnormalities in neurologically normal PKU patients remain unclear although abnormalities in VEPs which were not associated with the degree of MRI abnormalities in our sample indicate a disturbance in myelination along the visual pathways.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/PL00014255
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  • 4
    Electronic Resource
    Electronic Resource
    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
    Keywords: Node of Ranvier ; Sodium Permeability ; Aconitine
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: 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.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00586624
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  • 5
    Electronic Resource
    Electronic Resource
    Die Wirkung von Skorpiongift auf die Ionenströme des Ranvierschen Schnürrings (1968)
    Springer
    Pflügers Archiv 303 (1968), S. 133-149 
    Details
    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.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00592631
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  • 6
    Electronic Resource
    Electronic Resource
    Die Wirkung von Skorpiongift auf die Ionenströme des Ranvierschen Schnürrings (1968)
    Springer
    Pflügers Archiv 303 (1968), S. 150-161 
    Details
    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.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00592632
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  • 7
    Electronic Resource
    Electronic Resource
    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
    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.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00586041
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  • 8
    Electronic Resource
    Electronic Resource
    Gefüge und Korrosionsverhalten eines stickstofflegierten rostfreien Stahls (1998)
    Weinheim : Wiley-Blackwell
    Materialwissenschaft und Werkstofftechnik 29 (1998), S. 329-335 
    Details
    ISSN: 0933-5137
    Keywords: Chemistry ; Polymer and Materials Science
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics
    Description / Table of Contents: Microstructure and Corrosion Behaviour of a Nitrogen Alloyed Stainless SteelThe corrosion behaviour of a nitrogen alloyed stainless steel is investigated using potentiodynamic anodic polarization in physiological NaCl solution. The microstructure of forged samples is determined by microscopy and hardness measurements. The steel shows a globular austenitic structure. In the zone of large forging deformation the grains are elongated. Precipitates are observed in which chromium and molybdenium are enriched. The breakdown potentials range from 200 to 1170 mV vs. SCE, most of the values exceed 500 mV. The breakdown potentials decrease with increasing aspect ratio of the grains and increasing hardness. The precipitates are initial sites for pitting and crater shaped metal dissolution.
    Notes: Das Korrosionsverhalten eines stickstofflegierten rostfreien Stahles wird durch potentiodynamische anodische Polarisation in physiologischer NaCl-Lösung untersucht. Der Gefügezustand geschmiedeter Proben wird mikroskopisch und durch Härtemessungen ermittelt. Der Stahl weist ein globulares austenitisches Gefüge auf, in der Zone starker Schmiedeverformung sind die Körner gestreckt. Es liegen Ausscheidungen vor, in denen Chrom und Molybdän angereichert sind. Der Stahl zeigt Durchbruchspotentiale zwischen 200 und 1170 mV vs. SCE, die meisten Werte liegen über 500 mV. Die Durchbruchspotentiale fallen mit zunehmendem Streckungsgrad der Körner und zunehmender Härte ab. Die Ausscheidungen sind Initialpunkte für loch- und kraterförmigen Materialabtrag.
    Additional Material: 16 Ill.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1002/mawe.19980290704
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  • 9
    Electronic Resource
    Electronic Resource
    Modifikation von Titan durch Ionenimplantation-Ein Überblick (1997)
    Weinheim : Wiley-Blackwell
    Materialwissenschaft und Werkstofftechnik 28 (1997), S. 88-97 
    Details
    ISSN: 0933-5137
    Keywords: Chemistry ; Polymer and Materials Science
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics
    Description / Table of Contents: Modification of Titanium by Ion Implantation-A ReviewThis paper reviews investigations concerning the effect of ion implantation on the properties of titanium alloys. The correlations between the process parameters and the phase formation in implanted layers, the wear and strength properties as well as the corrosion and oxidation behaviour are described. The application for joint prostheses is mentioned.
    Notes: Es wird ein Überblick über den Einfluß der Ionenimplantation auf die Eigenschaften von Titanlegierungen gegeben. Die Beziehungen zwischen den Prozeßparametern und der Phasenbildung in ionenimplantierten Schichten, den Verschleiß- und Festigkeitseigenschaften sowie dem Korrosions- und Oxidationsverhalten werden dargestellt. Die Anwendung für Gelenkprothesen wird kurz besprochen.
    Additional Material: 6 Ill.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1002/mawe.19970280210
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  • 10
    Electronic Resource
    Electronic Resource
    Neue Sol-Gel-Beschichtungen als Korrosions-und Verschleißschutz für NE-Metalle (1998)
    Weinheim : Wiley-Blackwell
    Materialwissenschaft und Werkstofftechnik 29 (1998), S. 23-29 
    Details
    ISSN: 0933-5137
    Keywords: Chemistry ; Polymer and Materials Science
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics
    Description / Table of Contents: New Sol-Gel Based Coatings as Corrosion- and Wear-Protection On Non-Ferrous MetalsA new composite coating material has been developed for the protection of non-ferrous alloys (e.g. aluminum, magnesium, zinc and brass). The coating materials were prepared by the solgel process from epoxyalkoxysilanes and aromatic diols to act as crosslinking agents. As solvent, alcohols and glycol ethers have been used.The viscosity of the system (7-55 mPas) was established in a way to be used in spray, dip or flow coating processes. The transparent coatings were cured at T = 100-220°C. In corrosion tests, 3000 hours salt-spray climate and 240 hours CASS-test = Copper chloride and Acetic acid added Salt Spray, no infiltration of the metal-coating interface and no „filiform-corrosion“ on A199.5, AlMg3 and AlMgSil was observed. Brass samples showed less than 4 mm extension of a scribe after 500 hours salt-spray-test. The coatings showed no visible traces of abrasion after 1000 cycles taber abrader test and an excellent adhesion (cross cut and tape-test: grade 0). The transparent basic systems were pigmented by 5 - 10 wt.% of colored organic pigments to obtain all kind of colored coatings.
    Notes: Es wurde ein neuer Beschichtungswerkstoff auf Basis von anorganisch-organischen Kompositen zum Schutz von Metalloberflächen, besonders von Aluminium entwickelt. Das Beschichtungsmaterial wurde über den Sol-Gel-Prozeß synthetisiert, ausgehend von Epoxy-funktionalisierten Alkoxysilanen und aromatischen Diolen als Quervernetzer. Als Lösungsmittel wurden Alkohole und Glykolether verwendet.Die Viskosität der Systeme wurde so eingestellt, daß sowohl Sprüh-, als auch Tauch- und Flut-Beschichtungen durchgeführt werden konnten. Sie lag zwischen 7 und 55 mPas. Nach dem thermischen Verdichtungsprozeß bei 100-220°C wurden transparente Beschichtungen mit ausgezeichneter Haftung auf Al-Mg-, Zn-und Messing-Oberflächen (Gitterschnitt- und Tape-Test: Klasse 0) erhalten. Die Korrosionsfestigkeit beschichteter Aluminiumlegierungen (Al 99.5, AlMg3, AlMgSi I) wurde mit dem Salzsprühverfahren (DIN 50021) und dem CASS-Test (NaCl, CuCl2·2H2O, Essigsäure, pH=3) geprüft und auch nach 3000 Stunden trat keine Unterwanderung der Grenzfläche Metall / Schicht auf. Ebenso wurde keine „Filiform“-Korrosion beobachtet. Messingproben zeigten nach 500 h Salzsprühnebel-Test weniger als 4 mm mittlere Unterwanderungsbreite. Darüber hinaus wurde die Abriebbeständigkeit der Proben überprüft. Die Schichten zeigten keine sichtbare Beschädigung nach Prüfung des Abriebes mit dem Reibradverfahren (1000 Zyklen, Räder: CS 10-F).Es konnten stabile Dispersionen von organischen Farbpigmenten im transparenten Basissystem hergestellt werden, wodurch eine große Vielfalt farbiger Beschichtungen erhalten wurde.
    Additional Material: 7 Ill.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1002/mawe.19980290108
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