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Visualization of the Sugar Moiety in Lymphoid Cell Lines from Fabry’s Disease by Lectin Binding

  • A. Lageron
  • A. Negre
  • R. Salvayre

Abstract

Glycosphingolipid storage due to α-galactosidase deficiency (EC 3.2.1.22) characterizes the X-linked inherited Fabry’s disease (McKusick 30150). Accurate sugar moiety visualization obtained in kidney patients by lectin binding (Faraggiana et al., 1981; Lageron, 1987) prompted us to apply the same method to cell cultures. We chose to work on lymphoid cell lines (LC) obtained by Epstein-Barr virus transformation of lymphocytes, material often used in the study of genetic disease (Glade and Beratis, 1976), with the aim of determining what the lectin-binding method could bring to the knowledge of cell culture storage compared to other histochemical methods and whether modifications of the storage could be brought about by changes in the lipids in the culture medium.

Keywords

Sugar Moiety Lectin Binding Neuraminic Acid Fabry Patient Lymphoid Cell Line 
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References

  1. Bes, J. C., Salvayre, R., Caratero, C., Maret, A., Icart, J., Soleilhavoup, J. P. and Planel, H. Ultrastructural findings on an Epstein—Barr virus-transformed lymphoid cell line from a Fabry patient. Biol. Cell 47 (1983) 247–250Google Scholar
  2. Faraggiana, T., Churg, J., Grishman, E., Strauss, L., Prado, A., Bishop, D. F., Schuchman, E. and Desnick, R. J. Light and electron microscopic histochemistry of Fabry’s disease. Am. J. Pathol. 103 (1981) 247–262PubMedGoogle Scholar
  3. Glade, P. R. and Beratis, N. G. Long-term lymphoid cell lines in the study of human genetics. Progr. Med. Genet. 1 (1976) 1–48Google Scholar
  4. Lageron, A. Characterization by lectin binding of the sugar moiety of glycocompounds stored in inherited diseases. Histochem. J. 19 (1987) 419–425PubMedCrossRefGoogle Scholar

Copyright information

© SSIEM and Kluwer Academic Publishers 1989

Authors and Affiliations

  • A. Lageron
    • 1
  • A. Negre
    • 2
  • R. Salvayre
    • 2
  1. 1.INSERM U.55ParisFrance
  2. 2.INSERM U.101Hôpital PurpanToulouseFrance

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