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  • 1
    Electronic Resource
    Electronic Resource
    Histochemische Untersuchungen während der Wallerschen Degeneration bei C3H/An und C57bl-Mäusen (1969)
    Springer
    Cellular and molecular life sciences 25 (1969), S. 629-629 
    Details
    ISSN: 1420-9071
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary The reduction of the myelin sheath during the Wallerian degeneration is not delayed in C3H/An mice with reduced activity ofβ-d-glucuronidase. After section of the sciatic nerve, an increase of glucuronidase activity in Schwann's cells and macrophages can be shown here too.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01896556
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  • 2
    Electronic Resource
    Electronic Resource
    Histochemische Untersuchungen bei experimenteller Nervendurchtrennung (1965)
    Springer
    Naturwissenschaften 52 (1965), S. 592-593 
    Details
    ISSN: 1432-1904
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Chemistry and Pharmacology , Natural Sciences in General
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00631364
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  • 3
    Electronic Resource
    Electronic Resource
    Histochemische Untersuchungen zum Verteilungsmuster oxydativer Enzyme in Gliomen (1965)
    Springer
    Naturwissenschaften 52 (1965), S. 210-210 
    Details
    ISSN: 1432-1904
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Chemistry and Pharmacology , Natural Sciences in General
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00626469
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  • 4
    Electronic Resource
    Electronic Resource
    Nachweis und Verteilung der hydrolytischen Enzyme β-d-Glucuronidase, β-d-Galactosidase, β-d-Glucosidase und Arylsulfatase in intracraniellen Tumoren (1966)
    Springer
    Naturwissenschaften 53 (1966), S. 230-230 
    Details
    ISSN: 1432-1904
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Chemistry and Pharmacology , Natural Sciences in General
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00633904
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  • 5
    Electronic Resource
    Electronic Resource
    Histochemische Enzymnachweise in den neurosekretorischen Kernen der Ratte (1968)
    Springer
    Acta neuropathologica 10 (1968), S. 91-94 
    Details
    ISSN: 1432-0533
    Keywords: Neurosecretory nuclei ; Histochemistry ; Hydrolytic and oxydative enzymes
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung An den Gehirnen von 36 weiblichen Ratten wurden die Enzyme Cytochromoxydase, Lactat-, Succinodehydrogenase, DPNH- und TPNH-Diaphose, sowie β-Glucuronidase, β-Galactosidase, β-Glucosidase und Arylsulfatase in den neurosekretorischen Kernen untersucht. SDH und CyO zeigten eine niedrige Aktivität, während LDH, DPNH-Di und TPNH-Di deutlich nachweibsar waren. Die Reaktionen beim Glykosidennachweis fielen nur schwach aus, ein sicherer Nachweis der Arylsulfatase gelang nicht.
    Notes: Summary The enzymes cytochromoxydase, lactic-, succino-dehydrogenase, DPNH- and TPNH-diaphorase as well as β-glucuronidase, β-galactoidases, β-glucosidase and arylsulfatase were analysed in the paraventricular and supraoptic nucleus of the brains of 36 female rats. SDH and CyO showed a lower activity while LDH, DPNH-di and TPNH-di could be proved distinctly. On proving glycosidases the reactions were only moderate. A certain proof of arylsulfatase could not be furnished.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00690514
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  • 6
    Electronic Resource
    Electronic Resource
    Nachweis und Verteilung der Enzyme: β-d-Glucuronidase, β-d-Galaktosidase, β-d-Glucosidase und Arylsulfatase in Neurinomen (1969)
    Springer
    Acta neuropathologica 12 (1969), S. 189-194 
    Details
    ISSN: 1432-0533
    Keywords: Neurinomas ; Hydrolytic Enzymes ; Hydrolases ; Lipid Deposition ; Myelin Lipids
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung In 17 Neurinomen wurden Verteilung und Aktivität der hydrolytischen Enzyme β-d-Glucuronidase, β-d-Glucosidase, β-d-Galaktosidase und Arylsulfatase untersucht. Die höchste Aktivität der Enzyme zeigten die verfetteten Neurinome. Es bestand eine enge Beziehung zwischen Lipidablagerung und Fermentaktivität. Daraus wurde geschlossen, daß die im Neurinom gebildeten Markscheidenlipide unter Mitwirkung der untersuchten Hydrolasen abgebaut werden.
    Notes: Summary In 17 neurinomas, distribution and activity of the hydrolytic enzymes β-d-glucuronidase, β-d-glucosidase, β-d-galactosidase, and arylsulfatase were examined. Highest enzyme activity was seen in neurinomas stuffed with lipid material. There was close relationship between lipid deposition and enzyme activity. From these findings it was concluded that myelin lipids formed in neurinomas are degradated by means of the examined hydrolases.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00692506
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  • 7
    Electronic Resource
    Electronic Resource
    Verteilung und Aktivität hydrolytischer Enzyme in Hypophysenadenomen (1966)
    Springer
    Histochemistry and cell biology 7 (1966), S. 132-140 
    Details
    ISSN: 1432-119X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Description / Table of Contents: Zusammenfassung An 17 Hypophysenadenomen [12 Chromophobe (endokrin-inaktive), 5 Mischtypadenome (endokrin-aktive)] wurde das Verhalten und die Aktivität der hydrolytischen Enzyme β-Galaktosidase, β-Glucuronidase, β-Glykosidase und Arylsulfatase mit histochemischen Methoden geprüft. Die Mischtypadenome zeigen insgesamt eine höhere Aktivität als die Chromophoben. Dabei reagieren β-Galaktosidase und β-Glykosidase am stärksten, β-Glucuronidase etwas schwächer, während Arylsulfatase die niedrigste Aktivität zeigt. Die Befunde werden mit anderen enzymhistochemischen Untersuchungen an Hypophysenadenomen und tierexperimentellen Ergebnissen verglichen. Daraus folgt, daß wahrscheinlich zwischen der Aktivität der untersuchten lysosomalen Enzyme und der endokrinen Aktivität ein Zusammenhang besteht.
    Notes: Summary Behaviour and activity of the hydrolytic enzymes β-galactosidase, β-glucuronidase, β-glycosidase and arylsulphatase are tested in 17 adenomas of the hypophysis (12 chromophobic, endocrine-inactive; 5 mixed cell adenomas, endocrine-active). Mixed cell adenomas show an altogether higher activity than chromophobic adenomas. β-galactosidase and β-glycosidase show the highest, β-glucuronidase a slightly lower, and arylsulphatase the lowest activity. The findings are compared with other enzymhistochemical methods and results from animal experiments. The results of this comparison indicate that there is a correlation between the endocrine activity of the lysosomal enzymes in question.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00309718
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  • 8
    Electronic Resource
    Electronic Resource
    Glyceroltrioleate/glyceroltrierucate therapy in 16 patients with X-chromosomal adrenoleukodystrophy/ adrenomyeloneuropathy: effect on clinical, biochemical and neurophysiological parameters (1994)
    Springer
    European journal of pediatrics 154 (1994), S. 64-70 
    Details
    ISSN: 1432-1076
    Keywords: Key words     X-chromosomal adrenoleukodystrophy ; Adrenomyeloneuropathy ; Very long-chain fatty acids ; Therapy ; Peroxisomal disorder
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract      We have investigated the effect of glyceroltrioleate/glyceroltrierucate (GTO/GTE) therapy on X-chromosomal adrenoleukodystrophy in 16 patients with adrenoleukodystrophy (n = 6), adrenomyeloneuropathy (n = 3), Addison disease without neurological involvement (n = 2), and neurologically and endocrinologically asymptomatic patients (n = 5). Therapy was carried out for 19.4 ± 10 months. All patients showed a normalization of C 26:0 plasma fatty acid concentrations. None of the seven neurologically asymptomatic patients developed neurological symptoms. Somatosensory evoked potentials of the tibialis nerve was the most sensitive electrophysiological parameter, showing a slight improvement in neurologically asymptomatic patients during therapy. In none of the patients with normal cranial MRI at start of therapy (n = 6) has MRI deterioration been observed whilst on therapy. Follow up of the neurologically asymptomatic children supports the hypothesis that GTO/GTE therapy might prevent the development of neurological symptoms. Six of the nine neurologically symptomatic patients deteriorated to varying degrees whilst on therapy. MRI alterations have worsened in all patients with clinical deterioration. Conclusion     GTO/GTE treatment should be initiated in all neurological asymptomatic boys before first neurological symptoms develop. To discover these patients very long-chain fatty acid determination should be performed in all family members at risk when adrenoleukodystrophy or adrenomyeloneuropathy is diagnosed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01972976
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  • 9
    Electronic Resource
    Electronic Resource
    Isolated and combined deficiencies of NADH dehydrogenase (complex I) in muscle tissue of children with mitochondrial myopathies (1990)
    Springer
    European journal of pediatrics 150 (1990), S. 104-108 
    Details
    ISSN: 1432-1076
    Keywords: Reduced nicotinamide adenine dinucleotide (NADH) dehydrogenase ; Respiratory chain ; Mitochondrial myopathies
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We describe eight children with complex I deficiency, four of them with an isolated, the other four with an additional deficiency of complex IV. Clinical, chemical and morphological findings were compared from patients with isolated and combined deficiency. In both groups, the age of onset of symptoms was between the 1st day and the 4th month of life. Clinical and biochemical heterogeneity were observed. We found no correlation between residual activity of complex I in muscle, blood lactate level, and severity of clinical symptoms. Newborns presenting with severe lactic acidosis and children with later onset myopathy were seen in both groups. The group with combined complex I deficiency showed a more severe clinical course. By light microscopy ragged red fibres were only found in two patients with combined deficiency. However, by electron microscopy structural alterations of the mitochondria were observed in six out of seven muscle specimens.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02072049
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  • 10
    Electronic Resource
    Electronic Resource
    Simultaneous measurement, using flow cytometry, of radiosensitivity and defective mitogen response in ataxia telangiectasia and related syndromes (1992)
    Springer
    European journal of pediatrics 151 (1992), S. 756-760 
    Details
    ISSN: 1432-1076
    Keywords: Ataxia telangiectasia ; Radiosensitivity ; Immunodeficiency ; Flow cytometry ; Cell cycle
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In a retrospective study, peripheral blood mononuclear cells from 13 patients with known ataxia telangiectasia (AT) (Louis Bar syndrome, McKusick #20890) were irradiated with different doses of X-rays prior to stimulation with phytohaemagglutinin. Mitogen response and cell cycle progression were assessed by two-parameter 5-bromo-2′-deoxyuridine/Hoechst — ethidium bromide flow cytometry. Compared to age-matched controls, AT cells show a severely defective mitogen response in both unirradiated and irradiated cells. Following irradiation with 1.5 Gy, AT cells exhibit significantly greater accumulations of cells in the G2 phase of the first cell cycle than controls. The ratio between the number of cells accumulated in the first cycle G2 phase and the growth fraction provides a clear distinction between AT and control cultures. In addition, two patients with microcephaly, normal intelligence, immunodeficiency, chromosomal instability and risk for lymphoreticular malignancies (Seemanová syndrome) and two patients with the Nijmegen breakage syndrome (both syndromes are listed as McKusick #25126) also exhibit very poor mitogen response and moderately increased G2 phase accumulations after X-irradiation. The simultaneous assessment of radiosensitivity and mitogen response in a single cell kinetic assay provides a speedy and accurate classification of cells of AT and AT-related syndromes.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01959085
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