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  • Chemistry  (91)
  • D600  (3)
  • Interferon  (3)
  • Key words Phenylketonuria  (3)
  • 1
    ISSN: 1432-1440
    Keywords: Bone marrow transplantation ; Natural killer cells ; Interferon ; Cycloporin A ; Graft-versus-host disease ; Methotrexate
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Twenty-one patients with acute and chronic leukemia or severe aplastic anemia were studied for NK activity against a thymoma cell line (Thy 121) before and after allogeneic bone marrow transplantation. The means of the pretransplant and post-transplant levels did not differ from the mean of 134 NK determinations in 67 healthy donors. There was no correlation between pretransplant NK levels and the appearance of graft-versus-host disease. Three weeks following bone marrow transplantation, pretransplant NK levels were observed. The sensitivity of NK cells to interferon was the same as in normal donors both before and after bone marrow transplantation. In contrast to methotrexate, cyclosporin A inhibited NK activity in patients and controls in vitro. In vivo cyclosporin A treatment, however, did not decrease NK levels in bone marrow recipients.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 64 (1986), S. 1036-1040 
    ISSN: 1432-1440
    Keywords: Sinlge cell assay, modified ; Natural killer cells ; Interferon
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Even though the enhancement of the lyitc capacity and the kinetics of lysis of natural killer cells (NK) by interferon has been well documented, an increase of the target-effector cell binding percentage is still disputed. We, therefore, modified the Grimm-Bonavida single-cell assay so that 400 to 600 cells per individual determination could be reliably evaluated. Using this assay, which makes possible separate determination of effector-target cell binding and target lysis, we demonstrated that, in addition to lytic capacity, target-effector cell binding is also increased by preincubating NK with 100 to 1,000 IU interferon alpha 2 per 106 cells. Our data indicate that interferon alpha 2 induces pre-NK cells to bind target cells and that it activates these pre-NK cells to kill the targets.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-0584
    Keywords: HLA antigens ; Regulation in vivo ; Interferon ; Myeloproliferative syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Eighteen patients with myeloproliferative syndrome (14 with chronic myeloid leukemia, four with essential thrombocytosis) were investigated for modulation of HLA antigens on peripheral blood lymphocytes, monocytes, and hematopoietic precursors during IFNα therapy as a sign of potentially increased immune recognition of malignant cells. After 1 month of IFNα therapy, an increased number of monocytes and hematopoietic precursor cells, but not of lymphocytes, expressed HLADQ antigens. In addition, a strong induction of HLA class-I antigens was found on both hematopoietic progenitors and normal peripheral blood mononuclear cells. With daily injections of IFN in the first month of therapy stimulation continuously increased, suggested a major effect of IFNa on hematopoietic progenitors with sustained enhanced expression of HLA class-I antigens during differentiation of myelomonocytic cells. HLA class-I antigen expression was consistently augmented by IFNα in all patients, irrespective of their hematological response.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Pflügers Archiv 410 (1987), S. 75-82 
    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
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Pflügers Archiv 412 (1988), S. 390-396 
    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
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  • 6
    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
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  • 7
    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
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 155 (1996), S. S97 
    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
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    The journal of membrane biology 112 (1989), S. 185-192 
    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
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  • 10
    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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