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  • Chemistry  (3)
  • 5-Oxoprolin-uria Rett Syndrome  (1)
  • 1
    ISSN: 1432-1076
    Schlagwort(e): Key words Carbohydrate-deficient glycoprotein syndrome ; Gonadal dysgenesis ; 5-Oxoprolin-uria Rett Syndrome ; Thyroid dysfunction
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract Two female siblings, born to consanguineous parents, presented with a similar phenotype characterized by severe growth and developmental failure, dysmorphic features, thyroïd and gonadal dysfunction, autistic traits and hand stereotypes resembling Rett syndrome. In the elder patient, analysis of urinary organic acids disclosed a very high excretion of 5-oxoproline (4.2 to 8.1 mol/mol creatinine) and enzyme assays of leucocyte extracts revealed a profound deficiency of 5-oxoprolinase. However, normal urinary organic acid profiles were found in the younger child. In view of their distinct dysmorphic features and severe growth deficiency, these siblings cannot be considered as Rett Syndrome variants. The Dubowitz and carbohydrate-deficient glycoprotein syndromes were also excluded clinically and biochemically respectively. We conclude that these patients suffer from a hitherto undescribed autosomal recessive disorder, unrelated to the 5-oxoprolinase deficiency of the elder sib. Conclusion The present findings give evidence that 5-oxoprolinase deficiency is not associated with a distinct morbid phenotype.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 2
    Digitale Medien
    Digitale Medien
    New York, NY [u.a.] : Wiley-Blackwell
    Journal of Applied Polymer Science 37 (1989), S. 513-525 
    ISSN: 0021-8995
    Schlagwort(e): Chemistry ; Polymer and Materials Science
    Quelle: Wiley InterScience Backfile Collection 1832-2000
    Thema: Chemie und Pharmazie , Maschinenbau , Physik
    Notizen: A series of imidized acrylic polymers of varying structural composition generated by reaction of methylamine with poly(methyl methacrylate) were blended with a range of styrene/acrylonitrile or SAN copolymers (0-33% AN) and with poly(vinyl chloride). On the basis of glass transition behavior determined by differential scanning calorimetry, some but not all imidized acrylic structures were found to be miscible with PVC and with SAN copolymers within a limited window of AN levels. Acid functionality in the imidized acrylics appears to hinder their miscibility with SAN rather significantly and with PVC to a lesser extent. Miscible SAN blends showed lower critical solution temperature behavior whereas miscible blends with PVC did not up to the highest attainable temperatures. The composition factors that influence the phase behavior are described and interpreted in terms of possible mechanisms.
    Zusätzliches Material: 7 Ill.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 3
    Digitale Medien
    Digitale Medien
    Stamford, Conn. [u.a.] : Wiley-Blackwell
    Polymer Engineering and Science 29 (1989), S. 1215-1218 
    ISSN: 0032-3888
    Schlagwort(e): Chemistry ; Chemical Engineering
    Quelle: Wiley InterScience Backfile Collection 1832-2000
    Thema: Chemie und Pharmazie , Maschinenbau , Physik
    Notizen: Transparent dimethyl siloxane network polymers with refractive indices near 1.40 may be applied to fused silica fibers (n = 1.458) as they are drawn to produce plastic-clad silica (PCS) optical fibers. The evanescent tail of the light energy propagating in the core of such fibers extends into the silicone cladding, where it interacts with chemical species present in the polymer. If the silicone is doped with a dye, the absorption spectrum or fluorescence spectrum of the dye is reflected in the transmission spectrum of the fiber. Further, if the dye changes its absorption spectrum or fluorescence spectrum as a result of diffusion of a chemical species into the silicone, the change is detectable in the fiber output. The polymer material properties which determine the performance of these sensors are described, along with examples of sensors for ammonia and oxygen which utilize either color changing or fluorescent dyes.
    Zusätzliches Material: 8 Ill.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 4
    Digitale Medien
    Digitale Medien
    Hoboken, NJ : Wiley-Blackwell
    Journal of Biomedical Materials Research 15 (1981), S. 335-341 
    ISSN: 0021-9304
    Schlagwort(e): Chemistry ; Polymer and Materials Science
    Quelle: Wiley InterScience Backfile Collection 1832-2000
    Thema: Medizin , Technik allgemein
    Notizen: A comparison of 130 grafts done in 101 patients included 47 veins, 25 Johnson & Johnson dialdehyde-starch-tanned collagen prostheses, and 58 NCGT grafts that had been followed for a 40-month period. This study had documented the fact that in spite of frequent poor runoff, a useful result is provided by the NCGT graft apparently due in part to its built-in nonthrombogenic structure providing a cumulative patency of 73%. The patency rate was even superior with venous bypasses. Therefore, we still feel that the autogenous vein provides the most useful bypass conduit available in vascular reconstruction. In situations where a vein could not be used or was not available the NCGT graft offers an equally useful nonthrombogenic surface to maintain prolonged patency. Thus, collagen appropriately modified can provide a useful prosthesis in vascular reconstruction.
    Zusätzliches Material: 1 Ill.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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