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  • 1
    ISSN: 1432-0533
    Keywords: Severe sialidosis ; α-Neuraminidase deficiency ; Neuropathology ; Congenital ascites ; Nephrosialidosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A 56-day-old infant with α-neuraminidase deficiency, whose clinical features included severe edema of extremities and ascites which resembled those in severe infantile sialidosis, was autopsied. Perforation, whose pathogenesis was unclear, was found on the descending portion of the duodenum. Light and electron microscope studies showed that neurons in the cerebral and cerebellar corticies, and the thoracic spinal cord contained membrane-bound vacuoles but no membranous cytoplasmic bodies. Zebra bodies were found only in the neurons of the spinal cord. The neurons in the paraganglion and in the Auerbach's myenteric plexus were also distended with numerous membrane-bound vacuoles. Hepatocytes, endothelial cells and Kupffer cells in the liver and glomerular and tubular epithelial cells in the kidney were swollen with a number of vacuoles although the patient showed none of the clinical features of renal involvement. These pathological changes were similar to those in nephrosialidosis reported by Le Sec et al. [Arch Fr Pediatr 35:819–829 (1978)].
    Type of Medium: Electronic Resource
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  • 2
    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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  • 3
    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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  • 4
    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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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 76 (1988), S. 606-612 
    ISSN: 1432-0533
    Keywords: Macular mouse ; Menkes kinky hair disease ; Copper therapy ; Golgi study ; Purkinje cell
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: 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.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-1793
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology
    Notes: Abstract By means of scanning and transmission electron microscopy, the influence of the red tide species Chattonella antiqua was examined with respect to the surface ultrastructures of chloride cells of the yellowtail Seriola quinqueradiata. Conspicuous ultrastructural alterations occurred on the apical surface of these cells. The majority of chloride cells in the control gills showed an apical surface with numerous cellular extensions, while more than half the chloride cells affected by red tide organisms exhibited an apical surface with fewer and smaller extensions, a wrinkled apical surface, or a protruded apical surface. These ultrastructural alterations of chloride cell surface may be due to the partial disturbance of salinity by C. antiqua, and reflect the changes of the ion-transport function in yellowtail gills exposed to red tide water.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1089-7623
    Source: AIP Digital Archive
    Topics: Physics , Electrical Engineering, Measurement and Control Technology
    Notes: The soft x-ray continuum radiation in tokamak fusion test reactor (TFTR) low-density neutral-beam discharges can be much lower than its theoretical value obtained by assuming a corona equilibrium. This reduced continuum radiation is caused by an ionization equilibrium shift toward lower states, which strongly changes the value of the average recombination coefficient of metallic impurities γ¯, even for only slight changes in the average charge Z¯. The primary agent for this shift is the charge exchange between the highly ionized impurity ions and the neutral hydrogen, rather than impurity transport, because the central density of the neutral hydrogen is strongly enhanced at lower plasma densities with intense beam injection. In the extreme case of low-density, high neutral-beam power TFTR operation (energetic ion mode) the reduction in γ¯ can be as much as one-half to two-thirds. We calculate the parametric dependence of γ¯ and Z¯ for Ti, Cr, Fe, and Ni impurities on neutral density (equivalent to beam power), electron temperature, and electron density. These values are obtained by using either a one-dimensional impurity transport code or a zero-dimensional code with a finite particle confinement time. As an example, we show the variation of γ¯ and Z¯ in different TFTR discharges.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Woodbury, NY : American Institute of Physics (AIP)
    Applied Physics Letters 51 (1987), S. 877-879 
    ISSN: 1077-3118
    Source: AIP Digital Archive
    Topics: Physics
    Notes: A transverse mode stabilized GaAlAs laser diode which includes a ZnSe layer for the waveguide has been developed. The double heterostructure of the GaAlAs laser is formed by low-pressure metalorganic chemical vapor deposition (MOCVD), and a ZnSe layer is grown by adduct-source MOCVD in order to block the injection current and change the real refractive index in the lateral direction. The fundamental transverse mode oscillation of more than 15 mW is obtained with a low threshold current of 28 mA and a high quantum efficiency of 76%. An output power as high as 25 mW is achieved.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    [S.l.] : American Institute of Physics (AIP)
    Review of Scientific Instruments 57 (1986), S. 2151-2153 
    ISSN: 1089-7623
    Source: AIP Digital Archive
    Topics: Physics , Electrical Engineering, Measurement and Control Technology
    Notes: Metal impurity concentrations are measured by the pulse-height-analyzer (PHA) diagnostic from Kα x-ray peak intensities by use of an averaged excitation rate 〈σv〉. Low-Z impurity concentrations are inferred from the continuum enhancement (relative to a pure plasma) minus the enhancement due to metals. Since the PHA does not resolve lines from different charge states, 〈σv〉 is a weighted sum of rates; coronal equilibrium is usually assumed. The 〈σv〉 used earlier omitted the intercombination and forbidden lines from the dominant heliumlike state. The result was an overestimate of metals and an underestimate of low-Z impurities in cases where metals were significant. Improved values of 〈σv〉 using recent calculations for H-, He-, and Li-like Fe range from 10% to 50% larger than the earlier rates and yield metal concentrations in better agreement with those from vacuum ultraviolet (VUV) spectroscopy.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Journal of materials science 6 (1987), S. 1287-1290 
    ISSN: 1573-4811
    Source: Springer Online Journal Archives 1860-2000
    Topics: Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics
    Type of Medium: Electronic Resource
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