chondroitin-sulfates and Tay-Sachs-Disease

chondroitin-sulfates has been researched along with Tay-Sachs-Disease* in 2 studies

Other Studies

2 other study(ies) available for chondroitin-sulfates and Tay-Sachs-Disease

ArticleYear
Diagnosis of Tay-Sachs disease using radiolabelled chondroitin 6-sulphate-derived trisaccharides.
    Journal of inherited metabolic disease, 1983, Volume: 6, Issue:3

    Topics: alpha-L-Fucosidase; Chondroitin; Chondroitin Sulfates; Humans; Liver; Oligosaccharides; Tay-Sachs Disease; Trisaccharides; Tritium

1983
Impaired degradation of chondroitin sulfate in GM2-gangliosidosis.
    Clinical genetics, 1982, Volume: 22, Issue:4

    We have prepared a new radiolabeled substrate, derived from chondroitin 6-sulfate oligosaccharide, for the assaying of chondroitin sulfate degradation by beta-N-acetylgalactosaminidase. Using this substrate, we found a striking deficiency of beta-N-acetylgalactosaminidase activity in the cultured skin fibroblasts of patients with Sandhoff disease and Tay-Sachs disease. DEAE-cellulose chromatography at pH 6.0 revealed that both isoenzymes A and B of beta-N-acetylgalactosaminidases from normal human liver participated in the catabolism of chondroitin 6-sulfate. However, there were major differences in substrate specificity between isoenzyme A and isoenzyme B.

    Topics: beta-N-Acetylhexosaminidases; Cells, Cultured; Chondroitin; Chondroitin Sulfates; Fibroblasts; Hexosaminidases; Humans; Liver; Sandhoff Disease; Skin; Tay-Sachs Disease

1982