Hurler and Hunter syndromes: mutual correction of the defect in cultured fibroblasts.

Science (New York, N.Y.) ·Vol. 162 ·No. 3853 ·1968-11-01 ·Pages 570-2

Fratantoni JC, Hall CW, Neufeld EF

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.

MeSH Terms

Carbohydrate Metabolism, Inborn Errors Culture Techniques Fibroblasts/metabolism Genotype Glycosaminoglycans/metabolism Humans Intellectual Disability Molecular Biology Mucopolysaccharidosis I/metabolism Mucopolysaccharidosis II/metabolism Retinitis Pigmentosa/metabolism Sex Chromosomes Skin/cytology Sulfates/metabolism Sulfur Isotopes Trypsin

Chemicals

Glycosaminoglycans Sulfates Sulfur Isotopes Trypsin

Authors & Affiliations

3 authors, click to expand affiliations / ORCID

Fratantoni J C

Hall C W

Neufeld E F

Article Info

Journal

Science (New York, N.Y.)

Abbr.

Science

ISSN

0036-8075

DOI

10.1126/science.162.3853.570

Published

1968-11-01

Pages

570-2

Language

English

Region

United States

NLM ID

0404511

Subset

External Links

PubMed source DOI: 10.1126/science.162.3853.570 View journal details

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Hurler and Hunter syndromes: mutual correction of the defect in cultured fibroblasts.

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