Cytochemical and biochemical detection of intracellularly accumulated sialyl glycoconjugates in sialidosis and galactosialidosis fibroblasts with Macckia amurensis.
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.
Kotani
Masaharu
M
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.
Yamada
Hideo
H
Sakuraba
Hitoshi
H
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
D006001:Glycoconjugates; D037121:Plant Lectins; D019158:N-Acetylneuraminic Acid; D005690:Galactose