Hurler and Hunter Syndromes: Mutual Correction of the Defect in Cultured Fibroblasts
Joseph C. Fratantoni(National Institutes of Health), Clara W. Hall(National Institutes of Health), Elizabeth F. Neufeld(National Institutes of Health)
Cited by 441
Abstract
The biochemical defect of cultuired skin fibroblasts from Hurler or Hunter patients (faulty degradation of sulfated mucopolysaccharide, resulting in excessive intracellular accumulation) may be corrected if cells of these two genotypes are mixed with each other or with normal cells. The effect is mediated by substances released into the medium.
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