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Research Article| Volume 54, ISSUE 2, P181-187, May 1982

Neuraminidase activities in sialidosis and mucolipidosis

  • Masaru Kuriyama
    Correspondence
    Correspondence should be sent to Masaru Kuriyama, Third Department of Internal Medicine, Faculty of Medicine, Kagoshima University, 1208-1 Usuki-cho, Kagoshima, Japan.
    Footnotes
    Affiliations
    Department of Biochemistry and Metabolism, The Tokyo Metropolitan Institute of Medical Science, Honkomagome 3–18, Bunkyo-ku, Tokyo Japan
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  • Tadashi Miyatake
    Affiliations
    Department of Biochemistry and Metabolism, The Tokyo Metropolitan Institute of Medical Science, Honkomagome 3–18, Bunkyo-ku, Tokyo Japan

    Department of Neurology, Jichi Medical School, Tochigi Japan
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  • Misao Owada
    Affiliations
    Department of Pediatrics, Nihon University School of Medicine, Tokyo Japan
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  • Teruo Kitagawa
    Affiliations
    Department of Pediatrics, Nihon University School of Medicine, Tokyo Japan
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  • Author Footnotes
    ∗ Present address: Third Department of Internal Medicine, Faculty of Medicine, Kagoshima University, 1208-1 Usuki-cho, Kagoshima 890, Japan.
DOI:https://doi.org/10.1016/0022-510X(82)90180-0
Neuraminidase activities in sialidosis and mucolipidosis
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      Abstract

      Defects of neuraminidase activities towards sialyloigosaccharides in fibroblasts and leucocytes and enhanced excretion of sialyloligosaccharides in urine were shown in patients with adult type sialidosis with partial deficiency of β-galactosidase and cherry red spot-myoclonus syndrome. No differences in their neuraminidase residual levels and urinary excretion patterns on thin-layer chromatography were found between these two disorders. In mucolipidosis II and III patients, the neuraminidase activities towards sialyloligosaccharides were almost normal in leucocytes, although decreased in fibroblasts. The discrepancy of neuraminidase activities towards 2 →3 and 2 →6 sialyloligosaccharide isomers was not noticed in all cases.
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      Article info

      Publication history

      Accepted: October 7, 1981
      Received: September 24, 1981

      Footnotes

      ☆This work was supported by a grant from the Ministry of Education, Science and Culture of Japan.

      Identification

      DOI: https://doi.org/10.1016/0022-510X(82)90180-0

      Copyright

      © 1982 Published by Elsevier Inc.

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