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  • 1
    ISSN: 1432-1076
    Keywords: Key words Mitochondria ; Fatty acid oxidation ; LCHAD-deficiency ; Mitochondriopathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report on a boy who suffered from microcephaly, growth retardation, cardiomyopathy and hepatic dysfunction. When he had his first febrile infection at the age of 3 months he showed metabolic decompensation. Laboratory parameters and clinical features were compatible with a β-oxidation defect or a respiratory chain disorder. Measurement of β-oxidation enzymes showed long-chain 3-hydroxyacyl CoA dehydrogenase (LCHAD) deficiency; determination of respiratory chain complex activities revealed complete absence of complex I, II, III and IV activities in skeletal muscle and reduced activities of complexes II and IV in cultured fibroblasts, with secondary dysregulation of ATP synthase. The patient was found to be homozygous for the MTP:G1528 C mutation (LCHAD-deficiency). Conclusion This patient had LCHAD deficiency as his primary metabolic disorder, leading to secondary inhibition of respiratory chain enzymes by ‘toxic’ metabolites.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 153 (1994), S. 267-270 
    ISSN: 1432-1076
    Keywords: Cytochrome c-oxidase ; Mitochondriopathy ; Proximal tubular acidosis ; Leigh disease ; Muscular disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report on a boy who developed proximal renal tubular acidosis with loss of carnitine at the age of about 6 months. A few months later he began to suffer from progressive muscular weakness and neurological disturbances. Blood biochemistry showed elevated lactate and β-hydroxybutyrate with increased lactate/pyruvate and β-hydroxybutyrate/acetoacetate ratios. A high urinary excretion of lactate and citric acid cycle intermediates was found. These results indicated a defect of the mitochondrial respiratory chain. Analysis of biopsy material from skeletal muscle revealed low activities of all respiratory chain complexes. In muscle and fibroblasts cytochrome c-oxidase (complex IV) was absent. Despite high dose multi-vitamin therapy the boy died at the age of 30 months from central respiratory failure. At autopsy the neuropathological diagnosis of Leigh disease was made.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    New York, NY [u.a.] : Wiley-Blackwell
    Journal of Applied Polymer Science 27 (1982), S. 211-223 
    ISSN: 0021-8995
    Keywords: Chemistry ; Polymer and Materials Science
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Chemistry and Pharmacology , Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics , Physics
    Notes: The degradation mechanism of styrene-polyester copolymer was studied by various experimental techniques such as TGA, DTA, IR spectroscopy, pyrolysis gas chromatography, and GCMS (gas chromatography-mass spectrometry). It is concluded that, mainly, there are two first-order degradation reactions during thermal degradation in the presence of air. The first step involves the scission of crosslinks/weak links with liberation of free linear chains. The second degradation step involves random scission of the free linear chains into smaller fragments. The various fragments were identified by pyrolysis gas chromatography and by GCMS. In oxygen atmosphere, the polymer was found to obey first-order kinetics with a single rate constant. Apparently, due to presence of oxygen species at the degrading surface, the two rate constants obtained during thermal degradation reaction are altered in such a fashion as to give a single rate constant.
    Additional Material: 8 Ill.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Weinheim : Wiley-Blackwell
    Angewandte Makromolekulare Chemie 131 (1985), S. 187-194 
    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: Die strahlungsinduzierte Pfropfcopolymerization von Methylmethacrylat auf Nylon-Fasern wurde bei Raumtemperatur untersucht. Das Homopolymere wurde durch Soxhlet-Extraktion abgetrennt. Die Pfropfausbeute nahm mit der Strahlendosis von 0,1768 bis 0,7072 Mrad und mit der Monomerkonzentration zu. Die Zugabe von Kupfersulfat und Natriumlaurylsulfat verminderte die Bildung von Homopolymerem. Die Zahl der pro 100 eV absorbierter Energie gebildeten Pfropfzweige (Gb) und der Pfropfungsgrad (Gb) wurden berechnet. Ein Reaktionsmechanismus wird vorgeschlagen.
    Notes: The radiation-induced graft copolymerization of methyl methacrylate onto nylon fibers was investigated at room temperature. The homopolymer was separated by soxhlet extraction. The graft yield increases with increase of dose rate from 0.1768 to 0.7072 Mrad. The percentage of grafting increase with increasing monomer concentration. Addition of copper sulphate and a non-ionic surfactant, sodium lauryl sulphate, supresses the formation of homopolymer. The value of Gb, the number of branches per 100 eV of energy absorbed in the substrate polymer, and the value of α, the fraction of substrate polymer grafted, have been computed. A kinetic scheme has been suggested.
    Additional Material: 3 Ill.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    New York : Wiley-Blackwell
    Journal of Polymer Science: Polymer Chemistry Edition 16 (1978), S. 2715-2719 
    ISSN: 0360-6376
    Keywords: Physics ; Polymer and Materials Science
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Chemistry and Pharmacology
    Additional Material: 5 Ill.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    New York : Wiley-Blackwell
    Journal of Polymer Science: Polymer Chemistry Edition 17 (1979), S. 2649-2649 
    ISSN: 0360-6376
    Keywords: Physics ; Polymer and Materials Science
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Chemistry and Pharmacology
    Type of Medium: Electronic Resource
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