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  • 1
    Electronic Resource
    Electronic Resource
    GD3 expression by cultured human tumor cells of neuroectodermal origin (1989)
    Springer
    Acta neuropathologica 79 (1989), S. 317-325 
    Details
    ISSN: 1432-0533
    Keywords: Neuroectodermal tumor cells ; Monoclonal antibody ; Ganglioside ; GD3
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Seven monoclonal antibodies (mAbs) reactive with ganglioside II3(NeuAc)2-LacCer (GD3) were generated; four of these mAbs (DMAb-21, DMAb-22, DMAb-23, and DMAb-24) by immunizing mice with GD3 adsorbed to Salmonella minnesota and the remaining three (DMAb-7, DMAb-8, and DMAb-17) with melanoma line SK-MEL 28, which contains 1.4 nmol sialic acid of GD3 per mg protein. The specificities of the mAbs were defined by high-performance thin-layer chromatography (HPTLC) immunostain and solid-phase radioimmunoassay (SP-RIA) with a panel of purified gangliosides. DMAb-7 and DMAb-8 reacted with GD3, IV3(NeuAc)2nLcOse4Cer(3′,8′-LD1), and very weakly with IV3(NeuAc)2II3NeuAc-GgOse4Cer (GTla), but not with II3NeuAc-LacCer (GM3), II3NeuAcGgOse3Cer(GM2), II3NeuAc-GgOse4Cer(GM1), II3NeuAc, IV3NeuAcGgOse4Cer (GD1a), II3(NeuAc)2GgOse3(GD2), II3(NeuAc)2GgOse4Cer (GD1b), IV3NeuAcII3(NeuAc)2, GgOse4Cer(GT1b), suggesting the binding epitope to be a terminal tetrasaccharide NeuAcα2-8NeuAcα2-3Galβ1-4(Glc or GlcNAc). DMAb-7 and DMAb-8 were used to investigate the expression of GD3 on cultured human tumor cells of neuroectodermal origin. Thirteen of 19 gliomas, 3 of 5 medulloblastomas, 5 of 5 neuroblastomas, 2 of 2 melanomas, and 1 of 3 teratomas were shown to react with DMAb-8 and/or DMAb-7 by cell surface-RIA (CS-RIA) and immunofluorescence (IF) assays. HPTLC and densitometric analysis confirmed these results, as positive immunostains in the GD3 region were obtained with oligoganglioside fractions from 9 glioma, 1 medulloblastoma, 2 neuroblastoma, 1 melanoma, and 1 teratoma cell line. Glioma cell line U-105 MG and medulloblastoma cell line Daoy contain GD3 as shown by HPTLC immunostain analysis of extracts, although GD3 was undetectable on the cell surface as determined by CS-RIA and IF. There was no detectable GD3 found in gangliosides isolated from cell lines U-373 MG, D-54 MG, TE-671, and PA-1, which were negative for both DMAb-7 and DMAb-8 by CS-RIA and IF assay. Our results provide evidence that GD3 is expressed extensively with significant quantitative heterogeneity on cultured human neuroectodermal tumor cells including glioma, medulloblastoma, neuroblastoma, and melanoma.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00294668
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  • 2
    Electronic Resource
    Electronic Resource
    Late-infantile Gaucher disease in a child with myoclonus and bulbar signs: neuropathological and neurochemical findings (1991)
    Springer
    Acta neuropathologica 82 (1991), S. 152-157 
    Details
    ISSN: 1432-0533
    Keywords: Gaucher disease ; Myoclonus ; Epilepsy ; Gaze palsy ; Bulbar signs
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Clinical, neurochemical and neuropathological findings on a case of late-infantile Gaucher disease with oculomotor apraxia, progressive myoclonus and prominent bulbar signs are reported. There was a marked increase in glucosylceramide in cerebral cortex and cerebellum; the increase was more in the range of that seen in the Norrbottnian type III than in type II Gaucher disease. Cerebral cortical changes were characterized by a band-like intraparenchymal accumulation of Gaucher cells in lamina IV with an accompanying astrogliosis. In the cerebellum, a focal severe loss of granule cells and a global loss of dentate nucleus neurons was recorded. Milder changes were seen in thalamus and brain stem where perivascular accumulation of Gaucher cells was present in all regions. The cerebral cortical changes resembled those seen in type II Gaucher disease and was much more marked than in the Norrbottnian type III, whereas the changes in the dentate nucleus were more severe than in both type II and type III. The phenotypic variability with different patterns of clinical symptoms and neuropathological changes in neuronopathic Gaucher disease is discussed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00293959
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  • 3
    Electronic Resource
    Electronic Resource
    Chronic sclerosing panencephalitis (1970)
    Springer
    Acta neuropathologica 14 (1970), S. 284-292 
    Details
    ISSN: 1432-0533
    Keywords: Chronic Sclerosing Panencephalitis ; Myelin Involvement ; Lipid-Histochemistry ; Pathology of Oligodendroglia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Histologische und lipid-histochemische Untersuchungen wurden an zwei chronischen und einem subakuten Fall von sklerosierender Panencephalitis mit besonderer Berücksichtigung der Markschädigung durchgeführt. In den chronischen Fällen fanden sich fast völliger Markscheidenverlust, Reduktion der Oligodendroglia sowie zahlreiche Makrophagen. Histochemisch wurde in der Marksubstanz ein fast völliger Verlust von Cerebrosiden, Sphingomyelinen und Cholesterin nachgewiesen. Die Makrophagen enthielten vorwiegend Cholesterinester. Die Markscheiden des subakuten Falles erschienen größtenteils erhalten, doch fanden sich in der Oligodendroglia zahlreiche Kerneinschlüsse. Die Befunde werden mit den im Teil II mitgeteilten Ergebnissen der biochemischen Analyse verglichen. Es wird vermutet, daß die Schädigung der Oligodendroglia auch deren Fähigkeit, den Stoffwechsel der Markscheidenlipide aufrechtzuerhalten, beeinträchtigen dürfte. Der neurochemische Nachweis eines sehr niedrigen Gehaltes an Sphingolipiden, vor allem an Galaktolipiden, sowie deutlicher Veränderungen ihres Fettsäuregehaltes, selbst im subakuten Fall mit histologisch und histochemisch fast intakten Markscheiden, weisen auf eine frühe und tiefgreifende Störung des Stoffwechsels der Markscheidenlipide bei der sklerosierenden Panencephalitis hin.
    Notes: Summary A histological and lipid-histochemical study, with emphasis on myelin involvement, has been made of two chronic and one subacute case of sclerosing panencephalitis. An almost total loss of myelin sheaths was found in the brains of the chronic cases, accompanied by a reduction in the number of oligodendroglial cells and the occurrence of numerous compound granular corpuscles. Histochemically a practically complete disappearance of cerebrosides, sphingomyelins, and cholesterol was demonstrated in the white matter. The compound granular corpuscles mainly contained cholesteryl esters. The myelin sheaths of the subacute case appeared for the most part well preserved but numerous intranuclear inclusion bodies were observed in the oligodendroglia. The findings are discussed against the results of the biochemical analysis, presented in Part II. It might be supposed that the oligodendroglial lesions would also affect the capacity of the oligodendrocytes to maintain the metabolism of myelin lipids. In fact, the neurochemical observations of a very low content of sphingolipids, especially of galactolipids, and a marked change of their fatty acid composition, even in the subacute case with histologically and histochemically almost intact myelin sheaths, suggest an early and profound involvement in the metabolism of the myelin lipids in sclerosing panencephalitis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00685784
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  • 4
    Electronic Resource
    Electronic Resource
    Disialoganglioside GD2 in human neuroectodermal tumor cell lines and gliomas (1991)
    Springer
    Acta neuropathologica 82 (1991), S. 45-54 
    Details
    ISSN: 1432-0533
    Keywords: GD2 ; Monoclonal antibodies ; Gliomas ; Ganglisides ; Medulloblastomas
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Monoclonal antibodies (mAbs) recognizing the disialoganglioside II3(NeuAc)2GgOse3Cer (GD2) were produced by immunizing mice with the GD2-expressing neuroblastoma cell line LAN-1 and a prefusion boost with purified GD2 coupled to Salmonella minnesota. Two IgM mAbs were isolated which demonstrated high levels of reactivity (binding ratios in excess of 100) with GD2 by solid-phase radioimmunoassay and positivity in high-performance thin-layer chromatography (HPTLC) immunostain; only one (DMAb-20) was subsequently shown by analysis with a panel of defined ganglioside species to be specific for the minimum epitope of GD2, GalNAcβ1-4(NeuAcα2-8NeuAcα2-3)Gal-. DMAb-20 was used to evaluate the expression of GD2 by malignant glioma and medulloblastoma cell lines using cell surface radioimmunoassay, indirect membrane immunofluorescence, HPTLC immunostain, and densitometric analysis of extracted gangliosides from selected cell lines. Sixteen of 20 (80%) malignant glioma and 5 of 5 medulloblastoma cell lines reacted with DMAb-20; in agreement with previous studies, 5 of 5 neuroblastoma and 2 of 3 melanoma cell lines also reacted with DMAb-20. GD2 was proportionally increased in the glioma and medulloblastoma cell lines relative to levels in normal brain, as determined by densitometric analysis. In a phenotypic survey of malignant glioma biopsies, tumor cells in 24 of 30 (80%) cases stained positively with DMAb-20. Reactive astrocytes, both within and adjacent to tumors, were frequently intensely stained. Among the morphological variants of glioblastoma examined, the most intense staining with DMAb-20 was observed in neoplastic gemistocytes, with the weakest or absent staining in small cell glioblastomas. As GD2 is a commonly expressed surface antigen of gliomas and medulloblastomas, expression of which is retained in tissue culture, DMAb-20 will be useful in determining the functional role of GD2 in cell-cell interaction, adhesion, and invasion, and in defining altered growth control mechanisms of central nervous system neoplasms in in vitro models.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00310922
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  • 5
    Electronic Resource
    Electronic Resource
    Neuropathology of the Norrbottnian type of Gaucher disease (1984)
    Springer
    Acta neuropathologica 65 (1984), S. 99-109 
    Details
    ISSN: 1432-0533
    Keywords: Lipidosis ; Brain ; Human ; Gaucher disease ; Morphology ; Biochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The Norrbottnian type of Gaucher disease is characterized by infantile or juvenile onset and variable degrees of neurological symptoms, some of which develop only after splenectomy. A full neuropathological description of this type of Gaucher disease has not been reported previously. The brains of five patients were examined morphologically and biochemically. All presented typical accumulations of glucosylceramide storing cells in the adventitia of vessels in the cerebral and cerebellar sub-cortical white matter (s.c.w.m.). There were differences between the five cases with regard to the accumulation of adventitial storage cells and to the fatty acid pattern of the glucosylceramide isolated from the s.c.w.m., which implicate that the accumulation of glucosylceramide in adventitial cells in the brain is dependent on the generalized lipid storage process and enhanced by splenectomy. Loss of neurones and myelin was noted in the vicinity of accumulations of storage cells in two cases. The five cases whowed varying degrees of nerve cell loss, satellitosis and neuronophagia. Lipofuscin with simple and complex lipids but no glycolipids could be demonstrated in neurones light-microscopically. Utrastructural examination revealed inclusion bodies with bilayers in neurones of the cerebral and cerebellar cortex, dentate nucleus and pons. Because of the bilayered structure of Gaucher cell inclusions the bilayers in neurones are assumed to be formed by glucosylceramide. The fatty acid composition of glucosylceramide isolated from cerebral cortex in all cases suggested that cerebral gangliosides were its main precursor. The highest levels of psychosine (glucosylsphingosine) were seen in the cases with the most advanced nerve cell loss. The morphological and biochemical findings indicate that the neuronopathic process is associated with accumulation of glucosylceramide and psychosine in neurones.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00690463
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  • 6
    Electronic Resource
    Electronic Resource
    Chronic sclerosing panencephalitis (1970)
    Springer
    Acta neuropathologica 14 (1970), S. 293-303 
    Details
    ISSN: 1432-0533
    Keywords: Chronic Sclerosing Panencephalitis ; Myelin Involvement ; Lipid Composition ; Fatty Acid Composition ; Sphingolipids
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung In einem subakuten und zwei chronischen Fällen von sklerosierender Panencephalitis wurden der Lipidgehalt und das Fettsäuremuster des Gehirns untersucht. Bei den chronischen Fällen war der Gangliosidgehalt in der Hirnrinde leicht bis mäßig herabgesetzt. Bei allen Fällen fand sich im Mark ein frühzeitig auftretender und starker Verlust an Sphingolipiden, insbesondere Galaktolipiden. Die Fettsäurezusammensetzung der Phosphoglyceride war nur leicht gegenüber der Norm verändert. Die Cholesterinester enthielten sehr niedrige Konzentration an Polyensäuren und unterschieden sich damit grundsätzlich von jenen im normalen menschlichen Gehirn. Das Mark der beiden chronischen Fälle zeigte ein fast völliges Verschwinden von Cerebrosiden und Sulfatiden mit langkettigen Fettsäuren sowie eine relative Zunahme solcher mit mittel- bis langkettigen Fettsäuren und C23 h.
    Notes: Summary In one subacute and two chronic cases of sclerosing panencephalitis (SSPE), the lipid composition and the fatty acid pattern of cerebrum were studied. The ganglioside concentration in the cerebral cortex was slightly to moderately decreased in the chronic cases. In all the cases there was an early and extensive loss of sphingolipids, especially of galactolipids, in the white matter. The fatty acid composition of the phosphoglycerides was only slightly changed from the normal. The cholesteryl esters contained very low concentrations of the polyenoic acids and thus differ completely from those of normal human brain. In cerebral white matter of the two chronic cases there was an almost complete disappearance of cerebrosides and sulfatides with long-chain fatty acids and a marked relative increase of those with medium-short acids and C23 h.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00685785
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  • 7
    Electronic Resource
    Electronic Resource
    Ganglioside mapping of a human medulloblastoma xenograft (1989)
    Springer
    Acta neuropathologica 77 (1989), S. 283-288 
    Details
    ISSN: 1432-0533
    Keywords: Ganglioside ; Medulloblastoma ; Xenograft
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The ganglioside patterns of medulloblastomas have never been established; in this study we report the ganglioside profile of the human medulloblastoma cell line TE-671 grown as a xenograft in nude mice. Gangliosides were isolated and structurally analyzed by fast atom bombardment mass spectometry following permethylation. Identification of individual gangliosides was also performed by immunostaining of high-performance thin-layer chromatography-separated bands. Total ganglioside levels of 0.20 μmol/g of tissue were obtained, consistent with those reported for human glioma cell lines grown as xenografts; predominant monosialogangliosides of TE-671 xenografts were II3-α-NeuAc-LacCer (GM3) and II3-α-NeuAc-GgOse3 Cer (GM2) but there were also relatively large proportions of IV3-α-NeuAc-LcOse4Cer (3′-isoLM1), IV3-α-NeuAc-nLcOse4Cer (3′-LM1) and a further ganglioside of the neolactoseries with an extra lactosamine moiety. The only oligosialoganglioside detected was IV3, II3-α-NeuAc2-GgOse4Cer (GD1a).
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00687580
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  • 8
    Electronic Resource
    Electronic Resource
    Clinical, morphological, and biochemical investigations on a patient with an unusual form of neuronal ceroid-lipofuscinosis (1979)
    Springer
    European journal of pediatrics 132 (1979), S. 197-206 
    Details
    ISSN: 1432-1076
    Keywords: Neuronal ceroid-lipofuscinosis ; Serum lecithin pattern ; Arachidonic acid ; Linoleic acid ; Ultrastructural inclusions
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A patient with a progressive neurological disorder beginning at the age of three years is described. Mental and visual disturbances were the first signs, soon followed by ataxia and myoclonic jerks. Fundoscopy revealed a decreased pigmentation of the retina. Ultramicroscopic investigations of muscle and skin disclosed the typical changes seen in the late infantile and juvenile forms of neuronal ceroid-lipofuscinosis. In contrast to the clinical and ultrastructural findings, the fatty acid pattern of the serum lecithin showed a significant increase of arachidonic acid and a corresponding decrease of linoleic acid which is characteristic of the so-called infantile form of neuronal ceroid-lipofuscinosis (Hagberg-Santavuori variant; polyunsaturated fatty acid lipidosis). The obvious heterogeneity of the clinical, histological and laboratory findings within the subgroups of neuronal ceroidlipofuscinosis is briefly discussed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00442436
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  • 9
    Electronic Resource
    Electronic Resource
    Receptor ganglioside content of three hosts for sendai virus: MDBK, HeLa, and MDCK Cells
    Amsterdam : Elsevier
    Biochimica et Biophysica Acta (BBA)/Biomembranes 775 (1984), S. 7-16 
    Details
    ISSN: 0005-2736
    Keywords: Differentiation ; Gangliaside ; Paramyxocirus ; Virus receptor
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Chemistry and Pharmacology , Medicine , Physics
    Type of Medium: Electronic Resource
    URL: http://linkinghub.elsevier.com/retrieve/pii/0005-2736(84)90228-1
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  • 10
    Electronic Resource
    Electronic Resource
    Characterization of the binding epitope of the monoclonal antibody DMAb-1 to ganglioside GM2
    Amsterdam : Elsevier
    Biochimica et Biophysica Acta (BBA)/Lipids and Lipid Metabolism 1043 (1990), S. 267-272 
    Details
    ISSN: 0005-2760
    Keywords: Binding epitope ; GM2 derivative ; Ganglioside GM2 ; Monoclonal antibody ; TLC immunostaining
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Chemistry and Pharmacology , Medicine , Physics
    Type of Medium: Electronic Resource
    URL: http://linkinghub.elsevier.com/retrieve/pii/0005-2760(90)90026-T
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