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Science 27 August 1965:
Vol. 149. no. 3687, pp. 987 - 989
DOI: 10.1126/science.149.3687.987
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Articles

Hurler's Syndrome: Demonstration of an Inherited Disorder of Connective Tlssue in Cell Culture

B. Shannon Danes 1 and Alexander G. Bearn 1

1 The Rockefeller Ustiversity, New York 10021

Skin fibroblasts from three patients with Hurler's syndrome were grown in tissue culture and shown to contain metachromatic granules when stained for mucopolysaccharides with toluidine blue O. Similar inclusions were observed in cultures of fibroblasts from other members of the families, who appeared to be clinically normal but who were, judged from studies of pedigree, heterozygous or hemizygous for the abnormal gene.


THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES:
Ultrastructural Ocular Pathology of Hunter's Syndrome: Systemic Mucopolysaccharidosis Type II.
T. M. Topping, K. R. Kenyon, M. F. Goldberg, and A. E. Maumenee (1971)
Arch Ophthalmol 86, 164-177
   Abstract »    PDF »
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
   PDF »
Tay-Sachs Disease: Demonstration of the Stored Ganglioside in Cultured Cells From Brain Biopsy.
U. Batzdorf, L. L. Sarlieve, V. A. Gold, and J. H. Menkes (1969)
Arch Neurol 20, 650-652
   Abstract »    PDF »
Hurler and Hunter Syndromes: Mutual Correction of the Defect in Cultured Fibroblasts.
J. C. Fratantoni, C. W. Hall, and E. F. Neufeld (1968)
Science 162, 570-572
   Abstract »    PDF »
Mutant Enzymatic and Cytological Phenotypes in Cultured Human Fibroblasts.
J. G. Leroy and R. I. DeMars (1967)
Science 157, 804-806
   Abstract »    PDF »
Clinical Definition of the Hurler-Hunter Phenotypes: A Review of 50 Patients.
J. G. LEROY and A. C. CROCKER (1966)
Arch Pediatr Adolesc Med 112, 518-530
   Abstract »    PDF »
Vitamin C-Induced Increase of Dermatan Sulfate in Cultured Hurler's Fibroblasts.
I. A. Schafer, J. C. Sullivan, J. Svejcar, J. Kofoed, and W. V. B. Robertson (1966)
Science 153, 1008-1010
   Abstract »    PDF »


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Science. ISSN 0036-8075 (print), 1095-9203 (online)