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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Cellular and molecular life sciences 45 (1989), S. 126-129 
    ISSN: 1420-9071
    Keywords: L-gulono-γ-lactone oxidase ; ascorbic acid deficiency ; enzyme defect ; rat ; nuclei acid hybridization
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary A mutant strain of Wistar rats with L-gulono-γ-lactone oxidase deficiency has recently been established. To investigate this deficiency by DNA and RNA blot hybridization analyses, a fragment of a previously cloned cDNA encoding rat L-gulono-γ-lactone oxidase was used as a probe. When genomic DNA of the mutant rat was digested with several restriction enzymes, the probe hybridized to fragments of the same sizes as those produced from DNA of normal rats. Poly(A)+RNA from the liver of the mutant rat was found to contain an L-gulono-γ-lactone oxidase-specific mRNA of a normal size at a comparable level to that of normal rats. An in vitro translation experiment revealed that the mRNA programmed the synthesis of an enzyme protein which had the same molecular weight as that of the translational product of the normal mRNA, although the amount synthesized was markedly reduced as compared with that synthesized with the normal mRNA. In accordance with this observation, a very low but definite degree of L-gulono-γ-lactone oxidase activity was detected in the microsomes of the mutant rat by a newly developed, highly sensitive method.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0533
    Keywords: Menkes kinky hair syndrome ; Macular mottled mouse ; Mitochondrion ; Cytochromec oxidase ; Gold-labeling immuno-electron microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The macular mottled mouse is a murine model of the kinky hair syndrome, characterized by a deficiency in copper transport. Cytochromec oxidase (CCO), a respiratory enzyme, is located in the inner mitochondrial membrane and consists of seven subunits, along with copper and iron. Biochemical and histochemical findings indicated that CCO activity was decreased in the cerebellum of the macular mottled mice but not in that of the controls. Immunocytochemical analysis, using anti-CCO and anti-complex III rabbit sera, revealed that CCO in the macular mottled mice was stained more weakly than that in the controls. Immuno-electron microscopic examination of CCO and complex III, using a method of gold labeling, was also performed. In the control mice, a high concentration of gold particles present over CCO and complex III could be seen in the inner mitochondrial membrane. The number of CCO-labeled gold particles was remarkably less, however, in the macular mottled mice, while no significant difference was found in the labeling of complex III between the two groups. It may concluded that the very low CCO enzyme content in the macular mottled mouse results not only from a copper transport disorder but also from a CCO protein synthesis disorder which impairs the localization of CCO protein in the cerebellum.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Journal of thermal analysis and calorimetry 31 (1986), S. 547-551 
    ISSN: 1572-8943
    Source: Springer Online Journal Archives 1860-2000
    Topics: Chemistry and Pharmacology
    Description / Table of Contents: Zusammenfassung Durch Erweiterung der ursprünglichen Gleichung wird gezeigt, daß die Friedman-Methode zur Bestimmung der Aktivierungsenergie chemischer Reaktionen unter Verwendung sowohl der Konversion als auch der Geschwindigkeit in thermoanalytischen Daten breite Anwendungsmöglichkeiten auf dem Gebiet des Wachstums von Kristallen aus Keimen, der Diffusion und anderer aus nur einem Grundprozeß bestehenden Vorgänge bietet.
    Abstract: Резюме Расширением первона чального уравнения, показано, что метод Фр идмэна для установления энерги и активации химическ их реакций с использованием пара метров превращения и скорос ти в термоаналитичес ких данных широко применимо к пр оцессу роста кристаллов на основе существующих центро в кристаллизации, к про цессу диффузии и друг им процессам, в которых и меет место единствен ный процесс.
    Notes: Abstract By expanding the initial equation, it is shown that the Friedman method for estimating the activation energy of chemical reactions by using both the conversion and the rate in the thermoanalytical data has wide applicability to crystal growth from pre-existing nuclei, diffusion and other processes in which a single unit process is involved.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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