Abstract
Summary: Some of the serum proteins which bind to heparin and contribute to the pH 5.57 “heparin binding capacity” of human serum are glycoproteins; those from cystic fibrosis serum were found to be 27% higher in fucose (methylpentose) content, 27% lower in sialic acid content, and 31% lower in hexose content when compared to heparin-precipitated serum glycoproteins from normal control subjects. Hexosamine content of the heparin-precipitated serum glycoproteins was the same. Results of this preliminary investigation indicate that altered carbohydrate composition in serum glycoproteins may affect significantly their heparin binding capacity.
Speculation: The glycoprotein biosynthetic mechanism in cystic fibrosis is genetically altered and produces glycoproteins of abnormal carbohydrate composition which combine with normal, acidic, glycosaminoglycans to form insoluble, viscous complexes that hinder normal cellular and subcellular transport mechanisms and metabolic processes.
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Pearson, R., Lubin, A. Increased Heparin Binding in Cystic Fibrosis: A Reflection of Altered Glycoprotein Biosynthesis?. Pediatr Res 13, 834–840 (1979). https://doi.org/10.1203/00006450-197907000-00008
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DOI: https://doi.org/10.1203/00006450-197907000-00008
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