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  • 1
    Electronic Resource
    Electronic Resource
    Acylcarnitine profile in tissues and body fluids of biotin-deficient rats with and withoutl-carnitine supplementation (1994)
    Springer
    Journal of inherited metabolic disease 17 (1994), S. 678-690 
    Details
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Since biotin-deficient (BD) rats are a good animal model for human multiple carboxylase deficiency and have low plasma free carnitine levels, short-chain acylcarnitine profiles in biotin-deficient rats withl-carnitine supplementation (BDC rats) and BD rats were investigated by fast-atom bombardment and tandem mass spectrometry and gas chromatography/mass spectrometry. By the latter method, 3-hydroxyisovalerylcarnitine was identified in BD rats, and showed the greatest accumulation among short-chain acylcarnitines in tissues of BD rats, while the tissue levels of propionic acid were more markedly elevated than those of 3-hydroxyisovaleric acid. The tissue levels of 3-hydroxyisovaleryl-carnitine were significantly lower and those of propionyl-carnitine were somewhat higher in BDC rats than in BD rats, while the tissue levels of propionic acid and 3-hydroxyisovaleric acid in BDC rats were lower than those in BD rats. These changes were more apparent in kidney than in other tissues. The amounts of urinary excretion of acylcarnitines were markedly larger, and those of 3-hydroxyisovaleric acid were somewhat smaller in BDC rats than in BD rats, while those of propionic acid were very low in BD and BDC rats as compared with those of 3-hydroxyisovaleric acid. It seems that the relationship between the concentrations of 3-hydroxyisovalerylcarnitine and those of propionylcarnitine reflects the unique metabolism of the related metabolites in tissues, especially in kidney, which may be influenced by their urinary excretion and the availability of free carnitine. These data in biotin deficiency suggest that carnitine supplementation is possibly beneficial for patients with holocarboxylase synthetase deficiency who respond incompletely to biotin therapy.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00712010
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Effect of technological factors on the structure and properties of Eskaplen film material (1973)
    Springer
    Mechanics of composite materials 9 (1973), S. 5-9 
    Details
    ISSN: 1573-8922
    Source: Springer Online Journal Archives 1860-2000
    Topics: Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics
    Notes: Abstract The effect of the composition of the solvents, the concentration of the solutions, and the rate of film formation on the structure and properties of Eskaplen film material obtained on the basis of SKI-3 isoprene rubber hydrochloride has been investigated. The properties of the film material are shown to depend on the structure that develops during the film-forming process.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00856001
    Library Location Call Number Volume/Issue/Year Availability
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    Paper (German National Licenses)
    Fulltext
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