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  • Purkinje cell  (4)
  • Macular mouse  (3)
  • Microcephaly  (2)
  • 1
    ISSN: 1432-0533
    Schlagwort(e): Synapse ; Dentritic spine ; Purkinje cell ; Neuronal compensation
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Summary This study was undertaken to elucidate morphological changes in the synaptic area of the Purkinje cell dendritic spines when granule cells were decreased in number. The mice were injected s. c. with 30 mg/kg b. w. of cytosine arabinoside on days 2, 3, and 4, and on days 7, 8 and 9, and were designated as group I and group II, respectively. The mice injected with saline on days 2, 3, and 4 served as control. The cerebella of the mice in each group were examined by electron microscopy on days 30, 60, and 90. Using photographs thus obtained, the synaptic length and area of Purkinje cell dendritic spines which participated in synapses with axons of granule cells were measured by computer. In the controls, these spines did not increase significantly either in synaptic length or in spine area in the duration from 30 to 90 days after birth. In the 90-day-old mice belonging to group I and group II, however, they increased by about 20% in the synaptic length and by about 35% in the spine area as compared to those in age-matched control. The elongation and enlargement show that the synaptic surface on the spine spreads to compensate for synapses lost by reduction in number of granule cells in experimental groups.
    Materialart: Digitale Medien
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  • 2
    Digitale Medien
    Digitale Medien
    Springer
    Acta neuropathologica 59 (1983), S. 41-47 
    ISSN: 1432-0533
    Schlagwort(e): Cytosine arabinoside ; Cerebellum ; External granular layer ; Purkinje cell ; Heterotopic granule cell
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Summary This study was undertaken to elucidate the relationship between the time of destruction of the external granular layer and subsequent cerebellar abnormalities. Mice were injected s. c. with 30 mg/kg body weight (b. wt.) of cytosine arabinoside on days 2, 3, and 4, on days, 4, 5, and 6, on days 7, 8, and 9, and on days 10, 11, and 12, and designated as group I, II, III, and IV, respectively. In group I, disarrangement of Purkinje cells and heterotopic granule cells in the molecular layer were observed on all lobes of cerebellum. Heterotopic granule cells were seen on all lobes in group II, whereas disarrangement of Purkinje cells was observed only in the region from the anterior to middle lobes. In group III, heterotopic granule cells were limited to the area from anterior to middle lobes, but there was no disarrangement of Purkinje cells. Group IV cerebellum did not show abnormal cytoarchitecture. Golgi-Cox studies showed abnormal arborization of Purkinje cells in each experimental group. They were arbitrarily classified into inverted Purkinje cells, lying Purkinje cells, T-shaped Purkinje cells, and poorly arborized Purkinje cells. The earlier the postnatal treatment the more severe were the abnormalities of Purkinje cell dendrite. According to the electron-microscopic study, some glomerular synaptic complexes, which are normally confined to the internal granular layer, were observed even in the molecular layer in groups I, II, and III. Some of the Purkinje cell dentritic spines did not make synapses with parallel fibers in any of the experimental groups. The results indicate that severity of abnormal arborization of Purkinje cells is dependent on the period of destruction of the external granular layer. Formation of heterotopic granule cells was dependent on the destruction of the external granular layer up to day 10 after birth.
    Materialart: Digitale Medien
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  • 3
    Digitale Medien
    Digitale Medien
    Springer
    Acta neuropathologica 72 (1987), S. 349-354 
    ISSN: 1432-0533
    Schlagwort(e): Macular mutant mouse ; Menkes kinky hair disease ; Golgi study ; Purkinje cell ; Copper metabolism
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Summary This study was undertaken to elucidate, using the Golgi method, the neuropathological change in the brain of the macular mutant mouse, whose hemizygote (Ml/y) is considered to be a model of Menkes kinky hair disease (MKHD). The hemizygote mice gradually lost weight after 10 days of age and died with emaciation and seizure around day 15. The normal littermate (+/y) was well developed. In the cerebrum, the arborization of pyramidal neurons in the layer V of the Ml/y was the same as that in the +/y on day 10. However, development of arborization in the Ml/y was delayed in comparison with that in the +/y on days 12 and 14. Purkinje cells with several somal sprouts were observed in the cerebellum in both the Ml/y and +/y on day 7. The somal sprouts in the +/y had regressed gradually by day 12, while they were still in the anterior and middle lobes of the Ml/y on day 14. Additionally, the trunks of Ml/y stem dendrites became thicker and a cactus formation was recognized on the branching portion of the dendrites on day 14. Arborization of these abnormal Purkinje cells was distinctly poor compared with that in the +/y. These results suggest that the growth of the neurons is delayed in the Ml/y and simultaneously their cytoskeletal developments are disturbed, especially in the Purkinje cells. There is a close similarity in many respects to the neuropathological change in MKHD.
    Materialart: Digitale Medien
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  • 4
    ISSN: 1432-0533
    Schlagwort(e): Macular mouse ; Menkes kinky hair disease ; Copper therapy ; Mitochondrial abnormalities
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Summary The hemizygote of the macular mutant mice, which is clinically and neuropathologically considered to be a model of Menkes kinky hair disease (MKHD), were injected intraperitoneally four times with 10, 20, 20 and 30 μg of cupric chloride on days 4, 6, 8 and 10 after birth, respectively. Their cerebral and cerebellar cortices were chronologically examined by electron microscopy. In the cerebral cortes, only a few abnormal mitochondria with electron-lucent matrix and short peripherally located cristae were scattered in the neurons on day 14, and these had almost entirely vanished after day 21. In the cerebellar cortex, abnormal mitochondria were frequently found on day 14 in the dendrites of the Purkinje cells, whereas they were only occasionally observed in their cytoplasm. Those in the dendrites had decreased in number on day 30, and only a few of them were seen in the cerebellum after day 45. These results show that the copper therapy reduced ultrastructural abnormalities in the hemizygote of this mutant mouse.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 5
    ISSN: 1432-0533
    Schlagwort(e): Cytosine arabinoside ; Mice ; Microcephaly ; Cerebral cortex ; Hippocampus ; Abnormal cytoarchitecture
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Summary Pregnant mice were treated with cytosine arabinoside on days 13.5 and 14.5 of pregnancy. Brains of the offspring were studied histologically. The matrix layer of the embryonic brains was extensively destroyed 12h after the injection of cytosine arabinoside, but regenerated partially on day 17 of gestation. In the cerebral cortex of 1-, 3-, and 5-day-old treated mice, abnormal clusters of young neurons were found on the surface of the developing cerebral cortex. Some clusters still had a supply of immature neurons from the remnants of the regenerated matrix layer. After 20 days, the clusters became gradually indistinct, although some vestigial groups of neurons were observed even after 120 days. In the hippocampus of young mice, the pyramidal cells decreased in number and were disarranged. Heterotopic pyramidal cell masses were found in the stratum radiatum and in the molecular layer of the dentate gyrus. Apical dendrites of pyramidal cells exhibited abnormal arborization. It was demonstrated by3H-thymidine autoradiography that young neurons in the abnormal clusters in the cerebral cortex were those produced in the matrix layer regenerated after the destructive change by cytosine arabinoside.
    Materialart: Digitale Medien
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  • 6
    Digitale Medien
    Digitale Medien
    Springer
    Acta neuropathologica 76 (1988), S. 606-612 
    ISSN: 1432-0533
    Schlagwort(e): Macular mouse ; Menkes kinky hair disease ; Copper therapy ; Golgi study ; Purkinje cell
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Summary This study was undertaken to elucidate the clinical and neuropathological effects of copper administration on the macular mutant mouse. Its hemizygote, which is considered to be a model of Menkes kinky hair disease (MKHD), was injected intraperitoneally four times with 10, 20, 20 and 30 μg of cupric chloride on days 4, 6, 8 and 10, respectively. The hemizygote's curly whiskers gradually straightened and the frequent tonic seizures and ataxia disappeared after the injections. The body weight also gradually increased. In the cerebral cortex, the dendritic arborization of the pyramidal neurons in both the normal littermate and the treated hemizygote developed with time and reached the maximum around day 60. In the treated hemizygote, however, the arborization of the dendrites was significantly poor in comparison with that in the normal littermate from day 20 to 90. In the cerebellum of the treated hemizygote, the abnormal Purkinje cells with the few somal sprouts, thick stem dendrite and/or poor arborization, which were seen in the non-treated hemizygote, were improved by day 30, while their focal dendritic swellings remained even on day 60. These results indicate that the copper therapy improves not only the clinical manifestations but also the neuropathological changes, especially in the cerebellum.
    Materialart: Digitale Medien
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  • 7
    Digitale Medien
    Digitale Medien
    Springer
    Acta neuropathologica 72 (1987), S. 256-260 
    ISSN: 1432-0533
    Schlagwort(e): Macular mouse ; Menkes kinky hair disease ; Copper metabolism ; Mitochondrial abnormality ; Cerebrum
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Summary The macular mutant mouse was clinically and pathologically examined. The hemizygotes began to show white fur color and curly whiskers around postnatal day 3, then seizures and ataxia around day 8, while the normal littermates did not. The hemizygotes also increased weight gradually from birth to day 9, but then showed weight loss and died around day 15 with severe emaciation. These clinical features resembled those in Menkes kinky hair disease. There were no pathological changes in the cerebral cortex in the hemizygotes on day 7. On day 10, two to three clear vacuoles began to appear in a few neurons in the cerebrum. These neurons with vacuoles increased gradually in number and degenerative neurons were also observed by day 14. Ultrastructurally, they corresponded to giant abnormal mitochondria with an electron-lucent matrix and short peripherally located cristae. Other abnormal mitochondria, which were characterized by an electron-dense matrix with tubular or vesicular cristae, were also observed in the cerebral cortical neurons.
    Materialart: Digitale Medien
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  • 8
    Digitale Medien
    Digitale Medien
    Springer
    Acta neuropathologica 100 (2000), S. 403-408 
    ISSN: 1432-0533
    Schlagwort(e): Key words Cytosine arabinoside ; Heterotopia ; Microcephaly ; Hippocampus ; Immunohistochemistry
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract Pregnant mice were injected intraperitoneally with cytosine arabinoside (Ara-C) on days 13.5 and 14.5 of pregnancy. The brains of their offspring were studied histologically and histochemically. In addition to dysgenic microcephaly, nodular structures consisting of cells with a relatively homogeneous morphology were observed in the depths of the cerebral cortex. The cell clusters were first seen around postnatal day 4, and had a cellular continuity with the disarrayed pyramidal cell layer in the CA1 region of the hippocampus. Golgi-Cox staining showed a number of pyramidal-shaped cells in the clusters. Morphologically, they resembled the pyramidal neurons of the hippocampus. Immunohistochemical examination, using anti-serotonin or anti-tyrosine hydroxylase antibodies, also indicated similarities between the cell clusters and the pyramidal cell layer. It is, therefore, proposed that the cell clusters consisted of heterotopic pyramidal cells of the hippocampus. A few synaptic structures could already be detected in the heterotopic cell clusters on postnatal day 3 by electron microscopy. This early establishment of synaptic contact with related neurons may have caused the heterotopic localization of the pyramidal cells.
    Materialart: Digitale Medien
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