Metabolism of glycosaminoglycans in cultured normal and abnormal human fibroblasts

Abstract

Cultured fibroblasts from normal human embryos, Hurler's and Hunter's patients grown in the presence of 35S-sulfate, were harvested at different times and the amounts and specific activities of the “free” and “bound” intracellular and extracellular GAG were measured. Moreover, the incorporation of 35S (as cpm/mg dry cells) into the various GAG fractions was measured as an indication of the synthetic process. The patterns of incorporation of 35S into the intracellular “free GAG” of the three types of cells are similar to those originally described by Fratantoni et al. (1968), but differ considerably from the patterns of 35S incorporation into the intracellular “bound GAG” of the three types of cells. The data obtained indicate that the degradation of the intracellular “bound GAG” may not be defective in Hurler's or Hunter's cells.