1. The metabolism of the sulphated glycosaminoglycan fraction in cultured skin fibroblasts derived from a patient with the Hurler syndrome and from a normal subject was studied. Two labelled precursors, Na235SO4 and d-[2-3H]glucose, were used and their intracellular fates during uptake and chase' periods were assessed after separation of sulphated glycosaminoglycans from hyaluronic acid. After 4 or 8h of exposure to culture medium containing both labels, [35S]sulphate incorporation into the sulphated glycosaminoglycan fraction was twofold greater in Hurler-syndrome cells than in normal cells. At the same time, the rate of incorporation of [3H]glucose into the sulphated glycosaminoglycan fraction was approximately the same for both cell types. Consequently, an increased 35S/3H ratio (nmol of [35S]sulphate incorporated/nmol of [3H]glucose incorporated) was observed for Hurler-syndrome cells compared with normal cells. 2. The results of chase' experiments revealed that although the expected loss and relative retention of labelled sulphate occurred in the sulphated glycosaminoglycan fraction of normal and Hurler-syndrome cells, both cell types retained all of their radioactivity derived from [3H]glucose. 3. After 34h exposure to a corrective-factor' preparation from urine, the sulphated glycosaminoglycan content (as hexosamine and [35S]sulphate) of the Hurler-syndrome cells approached normal values. At the same time, there was an increase in specific radioactivity of corrected' Hurler-syndrome cells.

This content is only available as a PDF.
You do not currently have access to this content.