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  • 1
    ISSN: 1432-0533
    Keywords: Orthochromatic leukodystrophy (OLD) ; Globoid cell ; Proteolipid protein (PLP) ; Neuropathology ; Ceroid-lipofuscin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We report herein a sporadic case of the pigmentary type of orthochromatic leukodystrophy with early onset and very rapid clinical course. The patient's development was normal until 2 years old, when he experienced visual disturbance. Rapid deterioration resulted in death 1.5 years after the onset. Metachromatic leukodystrophy, globoid cell leukodystrophy and adrenoleukodystrophy were excluded by biochemical assays. Autopsy findings were compatible with the diagnosis of the pigmentary type of orthochromatic leukodystrophy. However, there were unique findings of severe neuronal loss and the collection of globoid-like cells in the interface of the gray matter and the white matter. Immunohistochemical staining of myelin basic protein, proteolipid protein and galactocerebroside demonstrated that these myelin constituents were equally preserved in the posterior column, while absent in the lateral and anterior columns of the spinal cord.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1435-1536
    Keywords: Chitosan ; molecular weight ; light scattering ; viscosity ; gel permeation chromatography
    Source: Springer Online Journal Archives 1860-2000
    Topics: Chemistry and Pharmacology , Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics
    Notes: Abstract The viscometric constantsa andK in the Mark-Houwink equation were determined in 0.5 M acetic acid-0.5 M.sodium acetate solution for chitosan fractionated by gel filtration. The weight-average molecular weight of each fraction was measured by the light-scattering method. The values obtained area=0.59 andK=0.119 cm3 g−1. The molecular weightsMw andMn for fractionated chitosan were measured by GPC. The value ofMw by GPC was much different from that by light scattering and, therefore, a universal calibration procedure was applied to the data by GPC. It was concluded that, also in the case of a cationic polysaccharide such as chitosan, the universal calibration procedure is effective for obtaining the reliable molecular weight by GPC.
    Type of Medium: Electronic Resource
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