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  • 1
    Electronic Resource
    Electronic Resource
    Nephrosialidosis: ultrastructural and lectin histochemical study (1993)
    Springer
    Acta neuropathologica 86 (1993), S. 198-205 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF00334891
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  • 2
    Electronic Resource
    Electronic Resource
    Pathological study on a severe sialidosis (α-neuraminidase deficiency) (1986)
    Springer
    Acta neuropathologica 71 (1986), S. 278-284 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF00688050
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  • 3
    Electronic Resource
    Electronic Resource
    A severe infantile sialidosis (β-galactosidase-α-neuraminidase deficiency) mimicking GM1-gangliosidosis type 1 (1983)
    Springer
    European journal of pediatrics 140 (1983), S. 295-298 
    Details
    ISSN: 1432-1076
    Keywords: β-Galactosidase ; α-Neuraminidase ; Sialidosis ; Nephrosialidosis ; GM1-Gangliosidosis type 1
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We observed a 3-month-old Japanese female infant with severe psychomotor retaration, coarse facial appearance, hepatosplenomegaly, and dysostosis multiplex. Only β-galactosidase was found to be deficient when the routine lysosomal hydrolase assay was performed on the patient's lymphocytes at 6 months of age. At first GM1-gangliosidosis type 1 seemed the most likely diagnosis. Later, however, additional studies (hydrolase assay in cultured skin fibroblasts, urinary oligosaccharide analysis, genetic complementation study, etc.) revealed that biochemical data of this case were in agreement with those of severe infantile sialidosis. The only important exception was that α-neuraminidase in the patient's lymphocytes showed normal activity but abnormal pH dependence toward 4-methylumbellyferyl substrate. In addition, a severely damaged kidney suggested that his case may be classified as a unique type of severe infantile sialidosis (possible nephrosialidosis). These observations stress the importance of careful biochemical diagnosis of a case with GM1-gangliosidosis type 1 phenotype.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00442667
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  • 4
    Electronic Resource
    Electronic Resource
    Degradation of hyaluronic acid at the metal surface (1998)
    Springer
    Colloid & polymer science 276 (1998), S. 388-394 
    Details
    ISSN: 1435-1536
    Keywords: Key words Hyaluronic acid ; degradation ; molecular weight ; metal surface ; hydroxyl radical
    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  When the viscosity of hyaluronic acid (HA) solution was measured with a rotational visco-meter, depolymerization occurred only by leaving the sample in the cup of the viscometer, while the solution in a glass test tube at the same temperature, 37 °C, was practically stable during the entire period of investigation. As the parts where the sample solution had contact with the viscometer are stainless steel, the participation of metal ions in the depolymerization was suspected. Therefore, experiments were carried out with stainless-steel beads in the HA solution. The results show that the molecular weight of HA decreased on exposure to the beads under aerobic conditions. The addition of 10 beads to 2 ml of a 0.1% HA solution prepared with 0.2 M NaCl reduced the molecular weight to about 75% of the initial value after 4 h. The degradation rate was proportional to the surface area of the beads. However, no dissolution of metal ion was detected, and therefore, this reaction was thought to proceed at the surface of the metal.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s003960050257
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  • 5
    Electronic Resource
    Electronic Resource
    Determination of the viscometric constants for chitosan and the application of universal calibration procedure in its gel permeation chromatography (1993)
    Springer
    Colloid & polymer science 271 (1993), S. 76-82 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF00652306
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