Summary
Secretion of lysosomal β-hexosaminidase by cultivated skin fibroblasts and receptor-mediated endocytosis of leucocyte β-hexosaminidase from a patient by cultivated non-parenchymal rat liver cells and skin fibroblasts were similar to that of a control proband. The results suggest normal oligosaccharide side chains of high mannose type on lysosomal enzymes in alpha1-antitrypsin (AAT) deficiency.
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References
Eagle H (1959) Amino acid metabolism in mammalian cell cultures. Science 130:432–437
Glasgow LR, Paulson JC, Hill R (1977) Systematic purification of five glycosidases from Streptococcus (Diplococcus) pneumoniae. J Biol Chem 252:8615–8623
Hasilik A, Neufeld EF (1980) Biosynthesis of lysosomal enzymes in fibroblasts. J Biol Chem 255:4937–4945
Hasilik A, Klein U, Waheed A, Strecker G, von Figura K (1980) Phosphorylated oligosaccharides in lysosomal enzymes: Identification of α-N-acetylglucosamine (1) phospho (6) mannose diester groups. Proc Natl Acad Sci USA 77:7074–7078
Hasilik A, Waheed A, von Figura K (1981) Enzymatic phosphorylation of lysosomal enzymes in the presence of UDP-N-acetylglucosamine. Absence of the activity in I-cell fibroblasts. Biochem Biophys Res Commun 98:761–767
Hercz A (1979) Studies on pronase glycopeptides and oligosaccharides from the liver Z variant of α1-antitrypsin. In: Schauer R et al. (eds) Glycoconjugates. Proceedings of the Fifth International Symposium, Kiel 1979. Thieme, Stuttgart, pp 143–144
Hickmann S, Neufeld EF (1972) A hypothesis for I-cell disease. Defective hydrolases that do not enter lysosomes. Biochem Biophys Res Commun 49:992–999
Kaplan A, Achord DT, Sly WS (1977) Phosphohexosyl components of a lysosomal enzyme are recognized by pinocytosis receptors on human fibroblasts. Proc Nat Acad Sci USA 74:2026–2030
Kornfeld R, Kornfeld S (1976) Comparative aspects of glycoprotein structure. Ann Rev Biochem 45:217–237
Montreuil J (1980) Primary structure of glycoprotein glycans. Basis for the molecular biology of glycoproteins. Adv Carbohydr Chem Biochem 37:157–223
Montreuil J (1982) Glycoproteins. Compr Biochem 19B:2
Morse JO (1978) Alpha1-antitrypsin deficiency. N Engl J Med 299:1045–1048
Snyder RA, Brady RO (1969) The use of white cells as a source of diagnostic material for lipid storage diseases. Clin Chim Acta 25:331–338
Tabas I, Kornfeld St (1978) The synthesis of complex type oligosaccharides. J Biol Chem 253:7779–7786
Tabas I, Kornfeld St (1979) Purification and characterization of a rat liver golgi α-mannosidase capable of processing asparagine-linked oligosaccharides. J Biol Chem 254:11655–11663
Ullrich K, Mersmann C, Weber E, von Figura K (1978) Evidence for lysosomal enzyme recognition by human fibroblasts via a phosphorylated carbohydrate moiety. Biochem J 170:643–650
Ullrich K, Mersmann G, Fleischer M, von Figura K (1978) Epithelial rat liver cells have cell surface receptors recognizing a phosphorylated carbohydrate on lysosomal enzymes. Hoppe-Seyler's Z Physiol Chem 359:1591–1598
Ullrich K, Gieselmann V, Mersmann G, von Figura K (1979) Endocytosis of lysosomal enzymes by non-parenchymal rat liver cells. Comparative study of lysosomal enzyme uptake by hepatocytes and non-parenchymal liver cells. Biochem J 182:329–335
von Figura K (1978) Secretion of β-hexosaminidase by cultivated human skin fibroblasts. Exp Cell Res 111:15–21
von Figura K, Weber E (1978) An alternative hypothesis of cellular transport of lysosomal enzymes in fibroblasts. Biochem J 176:943–950
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Ullrich, K. Normal exocytosis and endocytosis of lysosomal β-hexosaminidase in a case of alpha1-antitrypsin deficiency. Klin Wochenschr 61, 307–309 (1983). https://doi.org/10.1007/BF01497781
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DOI: https://doi.org/10.1007/BF01497781