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  • 1
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract— The effect of lipids other than the substrate cerebroside on the activity of cerebroside-sulphotransferase (CST) in Jimpy and normal mouse brain was investigated.The enzyme activity of an acetone-treated microsomal preparation can be stimulated in the presence of the extracted lipids either with or without addition of exogenous cerebroside as a substrate.The CST activity in the Jimpy mutant compared to that in normal animals differs from 18% in homogenate to approx 80% in solubilized or acetone-extracted microsomes.An addition of total lipid from normal mouse brain to microsomal preparations from which lipid has been removed by acetone results in a stimulation of Jimpy CST activity up to a value of 80% of normal mouse brain microsomes.Both Jimpy and normal mouse brain CST can be also stimulated by the addition of single lipid components such as cholesterol and lecithin by 50% in normal and 100% in Jimpy brain microsomes.These findings lead to the hypothesis that there is a lipid requirement for CST activity other than the substrate cerebroside.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Annals of the New York Academy of Sciences 540 (1988), S. 0 
    ISSN: 1749-6632
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Natural Sciences in General
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Annals of the New York Academy of Sciences 540 (1988), S. 0 
    ISSN: 1749-6632
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Natural Sciences in General
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    [s.l.] : Nature Publishing Group
    Nature 263 (1976), S. 436-438 
    ISSN: 1476-4687
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Chemistry and Pharmacology , Medicine , Natural Sciences in General , Physics
    Notes: [Auszug] Purified myelin basic protein was found to be an excellent substrate for the core-associated protein kinase (Figs 1 and 2a). No activator was needed, which suggests that myelin basic protein functions both as an activator and as a substrate4. The almost quantitative recovery of the radioactivity ...
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-0533
    Keywords: Key words Myelin-associated glycoprotein (MAG) ; Anti-MAG autoantibodies ; Paraproteinemic ; polyneuropathy ; Basal lamina ; Confocal microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report here on a patient with anti-myelin-associated glycoprotein (MAG) neuropathy in whom examination of a sural nerve biopsy by multichannel confocal microscopy showed a partly overlapping distribution of MAG and IgM deposits in myelinated fibers. Our data demonstrate that MAG in Schmidt-Lanterman incisures and paranodal loops, as well as some additional HNK-1-positive components of the basal lamina, are the major targets of the anti-MAG monoclonal IgM autoantibodies in this neuropathy in vivo. Perforation of the basal lamina can allow the penetration and binding of anti-MAG IgM inside myelinated fibers. Our results support and extend the notion that the production of monoclonal anti-MAG IgM may be antigenically driven by MAG molecules and that this process may occur in the immunologically privileged environment of the nerve prior to the appearance of a genuine gammopathy in serum.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-0533
    Keywords: Canine distemper encephalitis (CDE) ; Immunopathology ; Demyelination ; Immunoglobulin ; Local immune response
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary White matter lesions were induced in the brains of eight of nine dogs by means of experimental canine distemper virus (CDV) infection. Dogs were killed at 21, 24, 31, and 42 days after infection. Lymphocyte responsiveness to Con A and PHA stimulation in vitro was severely suppressed up to 31 days post infection (p.i.), followed by partial recovery as tested at 42 days p.i. Anti-CDV neutralizing antibody response was very weak in most dogs. There was a weak increase in antimyelin and antimyelin basic protein antibodies in most dogs during the course of the experiment. Dogs killed up to 31 days p.i. developed non-inflammatory demyclinating lesions in which no immunoglobulin could be detected. One of the three dogs that were killed at 42 days p.i. developed severe inflammatory demyelination. This was the only dog with a strong anti-MBP antibody response in the CSF and immunoglobulin demonstrated in demyelinating lesions. The present study supports previous observations that demyelination in acute CDV infection is not an immune mediated lesion but that these lesions may progress as a result of the local immune response. It is uncertain at this stage whether the local immune reaction specifically causes myelin destruction or whether bystander demyelination occurs in chronic CDE.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-0533
    Keywords: Canine distemper encephalitis (CDE) ; Immunopathology ; Demyelination ; Immunoglobulin ; Local immune response
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Nine dogs with canine distemper encephalitis (CDE) were examined with immunological techniques including demonstration of antibodies against canine distemper virus (CDV) in the serum and against myelin basic protein (MBP) in serum and in CSF. Mitogen stimulation tests of lymphocytes were also done. The brains were examined pathologically and immunoglobulin and C3 were demonstrated in lesions by means of immunohistological techniques. Six dogs with acute CDE had none or low antibody levels against CDV or MBP, and there was no immunoglobulin in demyelinating lesions. Some of these dogs had depressed lymphocyte mitogen responses. Two dogs with chronic CDE showed recovery of lymphocyte mitogen responses. One of these had a significant antibody response against CDV and MBP in the serum. Both dogs with chronic CDE had very high antibody titers against MBP in the CSF and demyelinating lesions contained immunoglobulin. These results suggest that acute demyelination in CDE is probably due to some direct viral activity and that the progression of demyelination in chronic CDE is associated with a local immune response.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 79 (1989), S. 160-167 
    ISSN: 1432-0533
    Keywords: Dysglobulinemia ; Myelin ; Myelin-associated glycoprotein ; Polyneuropathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Quantitative, immunopathological, light and electron microscopic studies of superficial peroneal nerve biopsies from 31 patients with IgM monoclonal gammopathy were carried out. Six patients had Waldenström's macroglobulinemia and 25 had IgM monoclonal gammopathy of undetermined significance. Serum samples from 28 of these patients were assayed for anti-myelin-associated glycoprotein (anti-MAG) activity. Anti-MAG activity was found in 25 of the samples. There was a relationship between the widening of some myelin lamellae observed on ultrastructural examination and the serum anti-MAG activity (23 cases). Immunopathological examination showed IgM binding to myelin sheaths in 17 cases.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Journal of neurology 225 (1981), S. 57-62 
    ISSN: 1432-1459
    Keywords: Myoclonus, postanoxic ; 5-Hydroxytryptophane ; 5-Hydroxyindolacetic acid ; Decarboxylase inhibitor ; Reflex loop
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Es wurde früher angenommen, daß der postanoxische Myoklonus durch eine Dysfunktion des ventro-lateralen thalamischen Nucleus bedingt ist. Diese Hypothese wurde durch verschiedene stereotaktische Studien in Frage gestellt. Neurochemische Untersuchungen, insbesondere die Messung von 5-Hydroxyindol-Essigsäure im Liquor öffnete den Weg zu neuen theoretischen und therapeutischen Möglichkeiten, Bahnen des serotoninergischen Systems zu beeinflussen. Ein typischer Fall wird vorgestellt, der unter einer Kombinationstherapie von 5-Hydroxytryptophan und einem Decarboxylase-Hemmer eine wesentliche Besserung zeigte. Die Pathogenese und therapeutische Möglichkeiten des postanoxischen Myoklonus werden diskutiert.
    Notes: Summary Postanoxic myoclonus was first accepted as being related to a dysfunction of the ventrolateral thalamic nucleus. Several stereotaxic studies have invalidated this hypothesis. The neurochemical approach, in particular the measure of 5-hydroxyindolacetic acid in the cerebrospinal fluid, has opened new theoretical and therapeutic possibilities involving serotoninergic pathways. A typical case is presented who improved markedly under a combined therapy with 5-hydroxytryptophan and a decarboxylase inhibitor. A review of the pathogenesis and therapeutic approach to postanoxic myoclonus is presented.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Journal of neurology 236 (1989), S. 353-355 
    ISSN: 1432-1459
    Keywords: Monoclonal IgM protein ; Neuropathy ; Gangliosides ; Enzyme-linked immunosorbent assay
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A patient with motor neuropathy associated with monoclonal IgM protein is reported. Using enzyme-linked immunosorbent assays, the antibody activity of the monoclonal igM was shown to be directed against GD1a ganglioside, a new and so far unreported specificity.
    Type of Medium: Electronic Resource
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