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Science 27 June 1969:
Vol. 164. no. 3887, pp. 1522 - 1523
DOI: 10.1126/science.164.3887.1522
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Articles

Glycolipid and Mucopolysaccharide Abnormality in Fibroblasts of Fabry's Disease

Reuben Matalon 1, Albert Dorfman 1, Glyn Dawson 2, and Charles C. Sweeley 2

1 Departments of Pediatrics and Biochemistry, Joseph P. Kennedy, Jr., Mental Retardation Center, LaRabida-University of Chicago Institute, University of Chicago, Chicago, Illinois
2 Department of Biochemistry, Michigan State University, East Lansing

Cultures of skin fiibroblasts from a patient with Fabry's disease showed an accumulation of the glycolipid, galactosyl-galactosyl-glucosyl ceramide. Such cells also showed metachromasia on staining with toluidine blue and a markedly elevated acid mucopolysaccharide content.


THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES:
Lactosyl Ceramidosis: Catabolic Enzyme Defect of Glycosphingolipid Metabolism.
G. Dawson and A. O. Stein (1970)
Science 170, 556-558
   Abstract »    PDF »
Genetic Inactivation of the agr-Galactosidase Locus in Carriers of Fabry's Disease.
G. Romeo and B. R. Migeon (1970)
Science 170, 180-181
   Abstract »    PDF »
Enzyme Replacement in Fabry's Disease, an Inborn Error of Metabolism.
C. A. Mapes, R. L. Anderson, C. C. Sweeley, R. J. Desnick, and W. Krivit (1970)
Science 169, 987-989
   Abstract »    PDF »


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