Vitamin C-induced increase of dermatan sulfate in cultured Hurler's fibroblasts.
In fibroblasts taken from patients witli Hurler's syndromne and grown in cultlure, dermatan sulfate constituted a larger percentage of the total sulfated glycosaminoglycans than it did in cultured fibroblasts from unaffected individuals. Moreover, the addition of ascorbic acid (vitamnin C) to the culture medium markedly increased the concentration of dermatan sulfate in the Hurler's fibroblasts but not in the normal fibroblasts. The biochemical phenotype of the Hurler's cells persisted during 28 weeks of serial culture.
D001205:Ascorbic Acid / Q000494:pharmacology*; D005347:Fibroblasts / Q000378:metabolism*; D006025:Glycosaminoglycans / Q000378:metabolism*; D006801:Humans; D066298:In Vitro Techniques; D008059:Mucopolysaccharidosis I / Q000378:metabolism*; D012867:Skin
D006025:Glycosaminoglycans; D001205:Ascorbic Acid