Cytochemical and biochemical detection of intracellularly accumulated sialyl glycoconjugates in sialidosis and galactosialidosis fibroblasts with Macckia amurensis

Clin Chim Acta. 2004 Jun;344(1-2):131-5. doi: 10.1016/j.cccn.2004.02.032.

Abstract

Background: To clarify the pathogenesis of and evaluate experimental therapeutic trials for lysosomal diseases, effective tools for the detection of intracellularly accumulated materials are required.

Methods: We examined a series of lectins for staining and blotting of the accumulated glycoconjugates in sialidosis and galactosialidosis.

Results: Lysosomally accumulated sialyl glycoconjugates were successfully detected in cultured fibroblasts from patients with these diseases by means of staining and blotting with Macckia amurensis (MAM).

Conclusions: This procedure is sensitive and easy, and will be useful not only for biochemical and diagnostic analyses, but also for therapeutic evaluation in these diseases.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Blotting, Western
  • Cells, Cultured
  • Clinical Laboratory Techniques
  • Fibroblasts / pathology*
  • Galactose
  • Glycoconjugates / analysis*
  • Humans
  • Lysosomal Storage Diseases / diagnosis
  • Maackia / chemistry
  • Mucolipidoses / diagnosis*
  • N-Acetylneuraminic Acid / analysis*
  • Plant Lectins*
  • Staining and Labeling

Substances

  • Glycoconjugates
  • Plant Lectins
  • N-Acetylneuraminic Acid
  • Galactose