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Science 26 August 1966:
Vol. 153. no. 3739, pp. 1008 - 1010
DOI: 10.1126/science.153.3739.1008
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Articles

Vitamin C-Induced Increase of Dermatan Sulfate in Cultured Hurler's Fibroblasts

Irwin A. Schafer 1, Julia C. Sullivan 1, Jiri Svejcar 1, Jorge Kofoed 1, and William Van B. Robertson 1

1 Department of Pediatrics, Stanford University School of Medicine, Palo Alto, California 94304

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.


THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES:
Inborn Errors of Mucopolysaccharide Metabolism: Faulty degradative mechanisms are implicated in this group of human diseases.
E. F. Neufeld and J. C. Fratantoni (1970)
Science 169, 141-146
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Science. ISSN 0036-8075 (print), 1095-9203 (online)