Abstract

Extracts of normal human livers and skin fibroblasts cultured from normal individuals and patients with the Hurler syndrome released L-iduronic acid when incubated with desulfated dermatan sulfate. Enzyme extracts of normal fibroblasts liberated larger amounts of L-induronic acid, as judged by paper chromatography, than did homogenates from Hurler fibroblasts. Preliminary studies with desulfated heparan sulfate using the same enzyme systems have also shown material with the R f of iduronolactone on paper chromatography.

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