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  • Digitale Medien  (3)
  • 1985-1989  (3)
  • Copper metabolism  (2)
  • Vacuole
Materialart
  • Digitale Medien  (3)
Erscheinungszeitraum
  • 1985-1989  (3)
Jahr
  • 1
    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
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 2
    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
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 3
    ISSN: 1615-6102
    Schlagwort(e): Budding ; Crystalloid ; 11 S globulin ; Protein body ; Pumpkin cotyledon ; Vacuole
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Biologie
    Notizen: Summary Vacuoles were isolated from pumpkin cotyledons at three developmental stages and judged to be pure by light microscopic inspection and marker enzyme assays. The time sequence of structural changes of vacuoles were examined by light microscopic inspection in parallel with their stainability with neutral red. Vacuoles isolated from the early stage of cotyledon development were heterogeneous in size (Ø=2–10 Μm) but stained uniformly with the dye. In contrast, vacuoles isolated from the middle stage were much larger (Ø=5–15 Μm), and there exist one to three cores, unstainable with neutral red, within a single vacuole. Electron microscopic observation confirms that vacuoles contain a few protein cores in cotyledon cells at the middle stage. Characteristically at this stage, it was observable that some large cores (Ø=4Μm) were budding from vacuoles. At the late stage, size of vacuoles becomes much smaller (Ø=6Μm), nearly equal to that of the protein bodies in dry seeds. Importantly, at this stage most of the volume of each vacuole was occupied by a single core, and only a small matrix space was stainable with neutral red. Suborganellar fractionation indicates that the vacuolar cores were identical to the crystalloids deposited in the protein bodies in dry seeds. Overall results strongly provide the evidence that one crystalloid buds from the vacuole during the later stage of seed maturation, giving rise to a protein body.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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