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  • Electronic Resource  (2)
  • 1990-1994  (2)
  • Nephrosialidosis  (1)
  • (Fluazifop)
  • Lipid peroxidation
  • Liposome
  • molecular weight
  • 1
    ISSN: 1432-0533
    Keywords: Nephrosialidosis ; Sialidosis ; α-neuraminidase deficiency ; Ultrastructure ; Lectin histochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The neuropathological findings in a Japanese male with nephrosialidosis are reported. Clinically, coarse face, psychomotor retardation, macular cherryred spot and proteinuria were noted at 1 year and 7 months. He was diagnosed to have nephrosialidosis on the basis of a deficiency of α-neuraminidase activity in both lymphocytes and cultured skin fibroblasts, and of severe glomerular and tubular involvement on renal biopsy. He died of multiple organ failure at 8 years and 6 months. There were numerous vacuoles and storage materials in visceral organs, particularly in the glomerular and tubular epithelial cells of the kidney and Kupffer cells as well as hepatocytes in the liver. Neuropathological examination revealed severe neuronal storage in the selected part of the central nervous system; lower motor neurons of the brain stem and spinal anterior horn cells, as well as neurons in the basal nucleus of Meynert. In the peripheral nervous system, sympathetic ganglia were severely affected. There was little or no neuronal storage in the basal ganglia, cerebral cortex or cerebellum, and demyelination was not found. Electron microscopic examination showed fine wavy multilamellar structures in the spinal anterior horn cells or Zebra body-like structures in the neurons of the Meynert's basal nucleus. Lectin histochemistry was positive for wheat germ agglutinin, Ricinus communis agglutinin-1 and peanut agglutinin within distended neurons. We conclude that the neuropathological feature in nephrosialidosis is not specific except for the selectiveness of the anatomical sites of involvement. It shares some aspects found in other types of sialidosis or galactosialidosis.
    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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