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  • 1985-1989  (5)
  • Menkes kinky hair disease  (3)
  • Brain damage  (1)
  • Cell kinetics  (1)
Material
Years
  • 1985-1989  (5)
Year
  • 1
    ISSN: 1432-069X
    Keywords: Cell kinetics ; Granulomas ; Homograft in athymic mice ; Mononuclear cells of athymic mice ; Local immunological control
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Schistosome egg granulomas in the livers of thymus-intact (nu/+) mice are large and contain eosinophils and mast cells, while those in nude athymic (nu/nu) mice are small and devoid of eosinophils or mast cells. To investigate the cell sources and cell kinetics of hepatic granulomas of nu/+ mice isolated and grafted into the skin of nu/nu mice, biopsies taken after grafting were examined by light and electron microscopy and autoradiography after 3H-thymidine (TdR) injection of either the donor or recipient mice. At 1 week, the grafted granulomas appeared to be amorphous and were surrounded by leukocytes, and the 3H-TdR-labeled donor cells had disappeared. After 2 weeks, repopulation with macrophages began and by 3–5 weeks, the granulomas morphologically resembled hepatic lesions of nu/+ mice. Injection of recipients with 3H-TdR before grafting, showed that labeled macrophages, eosinophils, and mast cells repopulated in granulomas. No granulomas were seen when nu/nu mice were grafted with schistosome eggs alone, and organ culture of nu/+ granulomas before grafting reduced the number of repopulated granulomas. These findings indicate that nu/+cells in grafted granulomas are replaced by nu/nu cells. Granulomatous reaction of the grafted sites in nu/ nu mice is influenced by a substance in nu/+ granulomas, and cells of nunu mice locally acquire a nu/+ type response.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 72 (1987), S. 349-354 
    ISSN: 1432-0533
    Keywords: Macular mutant mouse ; Menkes kinky hair disease ; Golgi study ; Purkinje cell ; Copper metabolism
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: 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.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-0533
    Keywords: Macular mouse ; Menkes kinky hair disease ; Copper therapy ; Mitochondrial abnormalities
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: 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.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 72 (1987), S. 256-260 
    ISSN: 1432-0533
    Keywords: Macular mouse ; Menkes kinky hair disease ; Copper metabolism ; Mitochondrial abnormality ; Cerebrum
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: 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.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-1920
    Keywords: Computed tomography ; Ventricular dilatation ; Colpocephaly ; Brain damage
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Cranial computed tomography (CT) of 108 cases with dilated lateral ventricles was reviewed to elucidate the relationship between focal vulnerability of developing brain and disproportional dilatation of lateral ventricles. CT findings of 108 cases with symmetrical dilatation of lateral ventricles were classified into three types by morphometry of lateral ventricles: anterior horn predominant type (31 cases), diffuse type (36 cases), posterior horn predominant type (41 cases). Posterior horn predominant type has a tendency to occur in congenital anomalies and premature brain damage, and anterior horn predominant type in infantile brain damage. This disproportional dilatation of anterior or posterior horns suggests a vulnerability of periventricular structure in developing brain.
    Type of Medium: Electronic Resource
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