Purification and characterization of sialic acid containing materials accumulated in cultured skin fibroblasts from a patient with type II sialidosis.
Sialyl conjugates which accumulate in sialidase deficient fibroblasts were made radioactive in the sialic acid moiety by culturing the cells in the presence of [3H]N-acetylmannosamine. The accumulated radioactive compounds were fractionated by anion exchange chromatography and gel filtration. At least nine chromatographically distinct sialyl conjugates were obtained. Neutral and amino sugar analysis of these compounds indicated that all were sialyloligosaccharides of the type which could be derived from glycoproteins containing complex, N-asparagine-linked carbohydrate groups.
D002478:Cells, Cultured; D005347:Fibroblasts / Q000032:analysis; D006595:Hexosamines / Q000378:metabolism; D006801:Humans; D009439:Neuraminidase / Q000172:deficiency*; D009844:Oligosaccharides / Q000302:isolation & purification*; D012867:Skin / Q000032:analysis; D014316:Tritium
D006595:Hexosamines; D009844:Oligosaccharides; C016735:sialooligosaccharides; D014316:Tritium; D009439:Neuraminidase; C002022:N-acetylmannosamine