title: Cytochemical and biochemical detection of intracellularly accumulated sialyl glycoconjugates in sialidosis and galactosialidosis fibroblasts with Macckia amurensis.
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.
authors.affiliation: Department of Clinical Genetics, The Tokyo Metropolitan Institute of Medical Science, Tokyo Metropolitan Organization for Medical Research, 3-18-22 Honkomagome, Bunkyo, Tokyo 113-8613, Japan.
authors.forename: Masaharu
authors.initials: M
authors.lastname: Kotani
authors.forename: Hideo
authors.initials: H
authors.lastname: Yamada
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Sakuraba
mesh_terms: D015153:Blotting, Western; D002478:Cells, Cultured; D019411:Clinical Laboratory Techniques; D005347:Fibroblasts / Q000473:pathology*; D005690:Galactose; D006001:Glycoconjugates / Q000032:analysis*; D006801:Humans; D016464:Lysosomal Storage Diseases / Q000175:diagnosis; D029901:Maackia / Q000737:chemistry; D009081:Mucolipidoses / Q000175:diagnosis*; D019158:N-Acetylneuraminic Acid / Q000032:analysis*; D037121:Plant Lectins; D013194:Staining and Labeling
chemical_list: D006001:Glycoconjugates; D037121:Plant Lectins; D019158:N-Acetylneuraminic Acid; D005690:Galactose