Skip to main content

Structure and Metabolism of Connective Tissue Proteoglycans

  • Chapter
The Biochemistry of Glycoproteins and Proteoglycans

Abstract

With the possible exception of hyaluronic acid, the connective tissue polysaccharides are all synthesized by their parent cells as components of proteoglycans. In these substances, a number of polysaccharide chains are covalently linked to a protein core; e.g., in the proteoglycan of bovine nasal cartilage, which is the prototype of molecules of this kind, close to 100 chondroitin sulfate chains, with a molecular weight of approximately 20,000, and slightly fewer keratan sulfate chains are linked to a core protein (mol. wt. 200,000) which constitutes 7–8% of the entire molecule. In many respects, the proteoglycans are similar to other protein-bound complex carbohydrates, and the conspicuous polysaccharide component per se does not distinguish the proteoglycans from the class of glycoproteins; e.g., there are members of the glycoprotein class, such as the blood group substances, which have a high relative content of carbohydrate consisting of a substantial number of monosaccharide units. Rather, the segregation of the proteoglycans into a separate category is based on a few specific characteristics: (1) each polysaccharide consists of repeating disaccharide units in which a hexosamine, d-glucosamine, or d-galactosamine is always present; (2) all connective tissue polysaccharides except keratan sulfate contain a uronic acid, either d-glucuronic acid or its 5-epimer, l-iduronic acid, or both; (3) ester sulfate groups are present in all members of the group except in hyaluronic acid; in addition, N-sulfate groups are found in heparin and heparan sulfate. Although certain other bipolymers are known to contain ester sulfate, e.g., some epithelial mucins (Horowitz, 1977), these compounds are clearly distinguishable from the connective tissue polysaccharides by the other criteria indicated above. It may also be mentioned that the d-glucuronic-acid-containing repeating disaccharide of chondroitin, N-acetylchondrosine, has recently been identified as a component of thyroglobulin (Spiro, 1977); however, since the disaccharide is present as a single unit, thyroglobulin may not be considered a proteoglycan.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

eBook
USD 16.99
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 109.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  • Amado, R., Ingmar, B., Lindahl, U., and Wasteson, Å., 1974, Depolymerisation and desulphation of chondroitin sulphate by enzymes from embryonic chick cartilage, FEBS Lett. 39:49.

    PubMed  CAS  Google Scholar 

  • Anseth, A., and Laurent, T. C., 1961, Studies on corneal polysaccharides. I. Separation, Exp. Eye Res. 1:25.

    PubMed  CAS  Google Scholar 

  • Antonopoulos, C. A., Engfeldt, B., Gardell, S., Hjertquist, S.-O., and Solheim, K., 1965, Isolation and identification of the glycosaminoglycans from fracture callus, Biochim. Biophys. Acta 101:150.

    PubMed  CAS  Google Scholar 

  • Antonopoulos, C. A., Axelsson, I., Heinegård, D., and Gardell, S., 1974, Extraction and purification of proteoglycans from various types of connective tissue, Biochim. Biophys. Acta 338:108.

    CAS  Google Scholar 

  • Arbisser, A. I., Donnelly, K. A., Scott, C. I., Di Ferrante, N., Singh, J., Stevenson, R. E., Aylesworth, A. S., and Howell, R. R., 1977, Morquio-like syndrome with beta galactosidase deficiency and normal hexosamine sulfatase activity: Mucopolysaccharidosis IVB, Am. J. Med. Gen. 1:195.

    CAS  Google Scholar 

  • Arbogast, B., Hopwood, J. J., and Dorfman, A., 1975, Absence of hyaluronidase in cultured human skin fibroblasts, Biochem. Biophys. Res. Commun. 67:376.

    PubMed  CAS  Google Scholar 

  • Arbogast, B., Hopwood, J. J., and Dorfman, A., 1977, Heparinase activity in rat liver, Biochem. Biophys. Res. Commun. 75:610.

    PubMed  CAS  Google Scholar 

  • Aronson, N. N., Jr., and Davidson, E. A., 1967a, Lysosomal hyaluronidase from rat liver. I. Preparation, J. Biol. Chem. 242:437.

    PubMed  CAS  Google Scholar 

  • Aronson, N. N., Jr., and Davidson, E. A., 1967b, Lysosomal hyaluronidase from rat liver. II. Properties, J. Biol. Chem. 242:441.

    PubMed  CAS  Google Scholar 

  • Aronson, N. N., Jr., and Davidson, E. A., 1968, Catabolism of mucopolysaccharides by rat liver lysosomes in vivo, J. Biol. Chem. 243:4494.

    PubMed  CAS  Google Scholar 

  • Bach, G., and Geiger, B., 1978, Human placental N-acetyl-β-d-hexosaminidase isozymes, Arch. Biochem. Biophys. 189:37.

    PubMed  CAS  Google Scholar 

  • Bach, G., Friedman, R., Weissmann, B., and Neufeld, E. F., 1972, The defect in the Hurler and Scheie syndromes: Deficiency of α-l-iduronidase, Proc. Natl. Acad. Sci. U.S.A. 69:2048.

    PubMed  CAS  Google Scholar 

  • Bach, G., Eisenberg, F., Cantz, M., and Neufeld, E. F., 1973, The defect in the Hunter syndrome: Deficiency of sulfoiduronate sulfatase, Proc. Natl. Acad. Sci. U.S.A. 70:2134.

    PubMed  CAS  Google Scholar 

  • Bäckström, G., Hallén, A., Höök, M., Jansson, L., and Lindahl, U., 1975, Biosynthesis of heparin, Adv. Exp. Med. Biol. 52:61.

    PubMed  Google Scholar 

  • Baker, J. R., and Caterson, B., 1977, The purification and cyanogen bromide cleavage of the link proteins from cartilage proteoglycan, Biochem. Biophys. Res. Commun. 77:1.

    PubMed  CAS  Google Scholar 

  • Baker, J. R., and Caterson, B., 1978, The isolation of “link proteins” from bovine nasal cartilage, Biochim. Biophys. Acta 532:249.

    PubMed  CAS  Google Scholar 

  • Baker, J. R., and Caterson, B., 1979, The isolation and characterization of the link proteins from proteoglycan aggregates of bovine nasal cartilage, J. Biol. Chem. 254:2387.

    PubMed  CAS  Google Scholar 

  • Baker, J. R., Cifonelli, J. A., and Rodén, L., 1969, The linkage of corneal keratosulphate to protein, Biochem. J. 115:11P.

    PubMed  CAS  Google Scholar 

  • Baker, J. R., Rodén, L., and Stoolmiller, A. C., 1972, Biosynthesis of chondroitin sulfate proteoglycan: Xylosyl transfer to Smith-degraded cartilage proteoglycan and other exogenous acceptors, J. Biol. Chem. 247:3838.

    PubMed  CAS  Google Scholar 

  • Baker, J. R., Cifonelli, J. A., and Rodén, L., 1975, The linkage of corneal keratan sulfate to protein, Connect. Tissue Res. 3:149.

    PubMed  CAS  Google Scholar 

  • Balasubramanian, A. S., and Bachhawat, B. K., 1964, Enzymic transfer of sulphate from 3′-phosphoadenosine 5′-phosphosulphate to mucopolysaccharides in rat brain, J. Neurochem. 11:877.

    PubMed  CAS  Google Scholar 

  • Balasubramanian, A. S., Joun, N. S., and Marx, W., 1968, Sulfation of N-desulfoheparin and heparan sulfate by a purified enzyme from mastocytoma, Arch. Biochem. Biophys. 128:623.

    PubMed  CAS  Google Scholar 

  • Barrett, A., 1972, in: Lysosomes (J. T. Dingle, ed.), p. 46, North-Holland, Amsterdam.

    Google Scholar 

  • Barrett, A., and Dingle, J. (eds.), 1971, Tissue Proteinases, North-Holland, Amsterdam.

    Google Scholar 

  • Barton, R. W., and Neufeld, E. F., 1971. The Hurler corrective factor: Purification and some properties, J. Biol. Chem. 246:7773.

    PubMed  CAS  Google Scholar 

  • Basner, R., Kresse, H., and von Figura, K., 1979, N-acetylglucosamine-6-sulfate sulfatase from human urine, J. Biol. Chem. 254:1151.

    PubMed  CAS  Google Scholar 

  • Beaudet, A. L., Di Ferrante, N. M., Ferry, G. D., Nichols, B. L., and Mullins, C. E., 1975, Variation in the phenotypic expression of β-glucuronidase deficiency, J. Pediatr. 86:388.

    PubMed  CAS  Google Scholar 

  • Beck, C., and Tappel, A. L., 1968, Rat-liver lysosomal β-glucosidase: A membrane enzyme, Biochim. Biophys. Acta 151:159.

    PubMed  CAS  Google Scholar 

  • Behrens, N. H., and Leloir, L. F., 1970, Dolichol monophosphate glucose: An intermediate in glucose transfer in liver, Proc. Natl. Acad. Sci. U.S.A. 66:153.

    PubMed  CAS  Google Scholar 

  • Behrens, N. H., Parodi, A. J., Leloir, L. F., and Krisman, C. R., 1971, The role of dolichol monophosphate in sugar transfer, Arch. Biochem. Biophys. 143:375.

    PubMed  CAS  Google Scholar 

  • Bell, C. E., Sly, W. S., and Brot, F. E., 1977, Human β-b-glucuronidase deficiency mucopolysaccharidoses: Identification of cross-reactive antigen in cultured fibroblasts of deficient patients by enzyme immunoassay, J. Clin. Invest. 59:97.

    PubMed  CAS  Google Scholar 

  • Bella, A., Jr., and Danishefsky, I., 1968, The dermatan sulfate-protein linkage region, J. Biol. Chem. 243:2660.

    PubMed  CAS  Google Scholar 

  • Berman, E. R., 1970, Proteoglycans of bovine corneal stroma, in: Chemistiy and Molecular Biology of the Intercellular Matrix (E. A. Balazs, ed.), p. 879, Academic Press, New York.

    Google Scholar 

  • Bhavanandan, V. P., and Meyer, K., 1968, Studies on keratosulfates: Methylation, desulfation, and acid hydrolysis studies on old human rib cartilage keratosulfate, J. Biol. Chem. 243:1052.

    PubMed  CAS  Google Scholar 

  • Bray, B. A., Lieberman, R., and Meyer, K., 1967, Structure of human skeletal keratosulfate, J. Biol. Chem. 242:3373.

    PubMed  CAS  Google Scholar 

  • Brimacombe, J. S., and Webber, J. M., 1964, Mucopolysaccharides, Biochim. Biophys. Acta Library, Vol. 6, Elsevier, Amsterdam.

    Google Scholar 

  • Brot, F. E., Bell, C. E., and Sly, W. S., 1978, Purification and properties of β-glucuronidase from human placenta, Biochemistry 17:385.

    PubMed  CAS  Google Scholar 

  • Cantz, M., and Kresse, H., 1974, Sandhoff disease: Defective glycosaminoglycan catabolism in cultured fibroblasts and its correction by β-N-acetylhexosaminidase, Eur. J. Biochem. 47:581.

    PubMed  CAS  Google Scholar 

  • Cantz, M., Kresse, H., Barton, R. W., and Neufeld, E. F., 1972, Corrective factors for inborn errors of mucopolysaccharide metabolism, Methods Enzymol. 28:884.

    Google Scholar 

  • Cashman, D. C., Laryea, J. U., and Weissmann, B., 1969, The hyaluronidase of rat skin, Arch. Biochem. Biophys. 135:387.

    PubMed  CAS  Google Scholar 

  • Caterson, B., and Baker, J. R., 1978, The interaction of link proteins with proteoglycan monomers in the absence of hyaluronic acid, Biochem. Biophys. Res. Commun. 80:496.

    PubMed  CAS  Google Scholar 

  • Caterson, B., and Baker, J. R., 1979, The link proteins as specific components of cartilage proteoglycan aggregates in vivo, J. Biol. Chem. 254:2394.

    PubMed  CAS  Google Scholar 

  • Cho, M. H., and Jaques, L. B., 1956, Heparinase. III. Preparation and properties of the enzyme, Can. J. Biochem. Physiol. 34:799.

    PubMed  CAS  Google Scholar 

  • Cifonelli, J. A., and King, J., 1972, The distribution of 2-acetamido-2-deoxy-d-glucose residues in mammalian heparins, Carbohydr. Res. 21:173.

    PubMed  CAS  Google Scholar 

  • Cifonelli, J. A., Ludowieg, J., and Dorfman, A., 1958, Chemistry of β-heparin (chondroitinsulfuric acid-B), J. Biol. Chem. 233:541.

    PubMed  CAS  Google Scholar 

  • Coppa, G. V., Singh, J., Nichols, B. L., and Di Ferrante, N., 1973, Urinary excretion of disulfated disaccharides in Hunter syndrome: Correction by infusion of a serum fraction, Anal. Lett. 6:225.

    CAS  Google Scholar 

  • Cöster, L., and Fransson, L.-Å., 1972, Isolation of proteoglycans from bovine sclera, Scand. J. Clin. Lab. Invest. 29(Suppl. 123):9.

    Google Scholar 

  • Dansihefsky, I., and Bella, A., 1966, The sulfated mucopolysaccharides from human umbilical cord, J. Biol. Chem. 241:143.

    Google Scholar 

  • Davidson, E. A., 1970, Glycoprotein and mucopolysaccharide hydrolysis (glycoprotein and mucopolysaccharide hydrolysis in the cell), in: Metabolic Conjugation and Metabolic Hydrolysis, Vol. 1 (W. H. Fishman, ed.), p. 327, Academic Press, New York.

    Google Scholar 

  • Dietrich, C. P., 1970, A heparin sulfamidase from mammalian lymphoid tissues, Can. J. Biochem. 48:725.

    PubMed  CAS  Google Scholar 

  • Dietrich, C. P., and De Oca, H. M., 1970, Production of heparin related mucopolysaccharides by mammalian cells in culture, Proc. Soc. Exp. Biol. Med. 134:995.

    Google Scholar 

  • Dietrich, C. P., and De Oca, H. M., 1978, Surface sulfated mucopolysaccharides of primary and permanent mammalian cell lines, Biochem. Biophys. Res. Commun. 80:805.

    PubMed  CAS  Google Scholar 

  • Dietrich, C. P., Silva, M. E., and Michelacci, Y. M., 1973, Sequential degradation of heparin in Flavobacterium heparinum:Purification and properties of five enzymes involved in heparin degradation, J. Biol. Chem. 248:6408.

    PubMed  CAS  Google Scholar 

  • Di Ferrante, N., Ginsberg, L. C., Donnelly, P. V., Di Ferrante, D. T., and Caskey, C. T., 1978, Deficiencies of glucosamine-6-sulfate or galactosamine-6-sulfate sulfatases are responsible for different mucopolysaccharidoses, Science 199:79.

    PubMed  Google Scholar 

  • Di Natale, P., Leder, I. G., and Neufeld, E. F., 1977, A radioactive substrate and assay for α-l-iduronidase, Clin. Chim. Acta 77:211.

    PubMed  Google Scholar 

  • Distler, J. J., and Jourdian, G. W., 1973, The purification and properties of β-galactosidase from bovine testes, J. Biol. Chem. 248:6772.

    PubMed  CAS  Google Scholar 

  • Dodgson, K. S., and Rose, F. A., 1970, Sulfoconjugation and sulfohydrolysis, in: Metabolic Conjugation and Metabolic Hydrolysis, Vol. 1 (W. H. Fishman, ed.), p. 239, Academic Press, New York.

    Google Scholar 

  • Dodgson, K. S., and Spencer, B., 1953, Studies on sulfphatases. I. The choice of substrate for the assay of rat-liver arylsulfatase, Biochem. J. 53:444.

    PubMed  CAS  Google Scholar 

  • Dodgson, K. S., Spencer, B., and Thomas, J., 1955, Studies on sulphatases. 9. The arysulfatases of mammalian livers, Biochem. J. 59:29.

    PubMed  CAS  Google Scholar 

  • Dorfman, A., 1969, Biosynthesis of acid mucopolysaccharides (glycosaminoglycans) of connective tissues, Thule International Symposia, Aging of Connective and Skeletal Tissue, p. 81, Nordiska Bokhandelns Förlag, Stockholm.

    Google Scholar 

  • Dorfman, A., 1974, Adventures in viscous solutions, Mol. Cell. Biochem. 4:45.

    PubMed  CAS  Google Scholar 

  • Dorfman, A., and Matalon, R., 1972, The mucopolysaccharidoses, in: The Metabolic Basis of Inherited Disease, 3rd ed. (J. B. Stanbury, J. B. Wyngaarden, and L. S. Fredrickson, eds.), p. 1211, McGraw-Hill, New York.

    Google Scholar 

  • Dorfman, A., and Matalon, R., 1976, The mucopolysaccharidoses (a review), Proc. Natl. Acad. Sci. U.S.A. 73:630.

    PubMed  CAS  Google Scholar 

  • Dorfman, A., Matalon, R., Cifonelli, J. A., Thompson, J., and Dawson, G., 1972, The degradation of acid mucopolysaccharides and the mucopolysaccharidoses, in: Sphingolipids, Sphingolipidoses and Allied Disorders (B. W. Volk and S. M. Aronson, eds.), p. 195, Plenum Press, New York.

    Google Scholar 

  • Dorfman, A., Arbogast, B., and Matalon, R., 1976, The enzymic defects in Morquio and Maroteaux-Lamy syndrome, in: Current Trends in Sphingolipidoses and Allied Disorders (B. W. Volk and L. Schneck, eds.), p. 261, Plenum Press, New York.

    Google Scholar 

  • Dulaney, J. T., and Moser, H. W., 1978, Sulfatide lipidosis: Metachromatic leukodystrophy, in: The Metabolic Basis of Inherited Disease (J. B. Stanbury, J. B. Wyngaarden, and D. S. Fredrickson, eds.), p. 770, McGraw-Hill, New York.

    Google Scholar 

  • Fabian, I., Bleiberg, I., and Aronson, M., 1976, Desulphation of heparin by mice and guinea pig leukocytes, Biochim. Biophys. Acta 437:122.

    PubMed  CAS  Google Scholar 

  • Faltynek, C. R., and Silbert, J. E., 1978, Copolymers of chondroitin 4-sulfate and chondroitin 6-sulfate in chick embryo epiphyses and other cartilage, J. Biol. Chem. 253:7646.

    PubMed  CAS  Google Scholar 

  • Fisher, D., and Kent, P. W., 1969, Rat liver β-xylosidase, a lysosomal membrane enzyme, Biochem. J. 115:50.

    Google Scholar 

  • Fisher, D., Higham, M., Kent, P. W., and Pritchard, P., 1966, β-Xylosidases of animal and other sources in relation to the degradation of chondroitin sulphate-peptide complexes, Biochem. J. 98:46P.

    CAS  Google Scholar 

  • Fluharty, A. L., Stevens, R. L., Sanders, D. L., and Kihara, H., 1974, Arylsulfatase B deficiency in Maroteaux-Lamy syndrome cultured fibroblasts, Biochem. Biophys. Res. Commun. 59:455.

    PubMed  CAS  Google Scholar 

  • Fluharty, A. L., Stevens, R. L., Fung, D., Peak, S., and Kihara, H., 1975, Uridine diphospho-N-acetylgalactosamine-4-sulfate sulfohydrolase activity of human arylsulfatase B and its deficiency in the Maroteaux-Lamy syndrome, Biochem. Biophys. Res. Commun. 64:955.

    PubMed  CAS  Google Scholar 

  • Foley, T., and Baker, J. R., 1973, Heparan sulphate sulphotransferase: Properties of an enzyme from ox lung, Biochem. J. 135:187.

    PubMed  CAS  Google Scholar 

  • Forsee, W. T., Belcher, J., and Rodén, L., 1978, Biosynthesis of heparin. The N-acetyl-glucosaminyl transfer reaction, Fed. Am. Soc. Exp. Biol. Fed. Proc. 37:1777.

    Google Scholar 

  • Fransson, L.-Å., 1968a, Structure of dermatan sulfate. III. The hybrid structure of dermatan sulfate from umbilical cord, J. Biol. Chem. 243:1504.

    PubMed  CAS  Google Scholar 

  • Fransson, L.-Å., 1968b, Structure of dermatan sulfate. V. The hybrid structure of dermatan sulfate from hog intestinal mucosa, Ark. Kemi 29:95.

    CAS  Google Scholar 

  • Fransson, L.-Å., and Havsmark, B., 1970, Structure of dermatan sulfate. VII. The copolymeric structure of dermatan sulfate from horse aorta, J. Biol. Chem. 245:4770.

    PubMed  CAS  Google Scholar 

  • Fransson, L.-Å., and Malmström, A., 1971, Structure of pig skin dermatan sulfate. 1. Distribution of d-glucuronic acid residues, Eur. J. Biochem. 18:422.

    PubMed  CAS  Google Scholar 

  • Fransson, L.-Å., and Rodén, L., 1967a, Structure of dermatan sulfate. I. Degradation by testicular hyaluronidase, J. Biol. Chem. 242:4161.

    PubMed  CAS  Google Scholar 

  • Fransson, L.-Å., and Rodén, L., 1967b, Structure of dermatan sulfate. II. Characterization of products obtained by hyaluronidase digestion of dermatan sulfate, J. Biol. Chem. 242:4170.

    PubMed  CAS  Google Scholar 

  • Fransson, L.-Å., Anseth, A., Antonopoulos, C. A., and Gardell, S., 1970, Structure of dermatan sulfate. VI. The use of cetylpyridinium chloride-cellulose microcolumns for determination of the hybrid structure of dermatan sulfates, Carbohydr. Res. 15:73.

    CAS  Google Scholar 

  • Fransson, L.-Å., Cöster, L., Havsmark, B., Malmström, A., and Sjöberg, I., 1974, The copolymeric structure of pig skin dermatan sulphate. Isolation and characterization of l-idurono-sulphate-containing oligosaccharides from copolymeric chains, Biochem. J. 143:379.

    PubMed  CAS  Google Scholar 

  • Friedman, R. B., and Weissmann, B., 1972, The phenyl α-and β-l-idopyranosid uronic acids and some other aryl glycopyranosiduronic acids, Carbohydr. Res. 24:123.

    PubMed  CAS  Google Scholar 

  • Friedman, Y., and Arsenis, C., 1972, The resolution of aryl sulfatase and heparin sulfamidase activities from various rat tissues, Biochem. Biophys. Res. Commun. 48:1133.

    PubMed  CAS  Google Scholar 

  • Friedman, Y., and Arsenis, C., 1974, Studies on the heparin sulphamidase activity from rat spleen: Intracellular distribution and characterization of the enzyme, Biochem. J. 139:699.

    PubMed  CAS  Google Scholar 

  • Fukuda, M., Muramatsu, T., Egami, F., Takahashi, N., and Yasuda, Y., 1968, Purification of β-xylosidase and its action on O-β-xylosyl l-serine and stem bromelain glycopeptide, Biochim. Biophys. Acta 159:215.

    PubMed  CAS  Google Scholar 

  • Galligani, L., Hopwood, J., Schwartz, N. B., and Dorfman, A., 1975, Stimulation of synthesis of free chondroitin sulfate chains by β-d-xylosides in cultured cells, J. Biol. Chem. 250:5400.

    PubMed  CAS  Google Scholar 

  • Ganschow, R. E., 1973, The genetic control of acid hydrolases, in: Metabolic Conjugation and Metabolic Hydrolysis, Vol. 3 (W. H. Fishman, ed.), p. 189, Academic Press, New York.

    Google Scholar 

  • Glaser, J. H., and Conrad, H. E., 1979, Chondroitin SO4 catabolism in chick embryo chondrocytes, J. Biol. Chem. 254:2316.

    PubMed  CAS  Google Scholar 

  • Glaser, L., and Brown, D. H., 1955, The enzymatic synthesis in vitro of hyaluronic acid chains, Proc. Natl. Acad. Sci. U.S.A. 41:253.

    PubMed  CAS  Google Scholar 

  • Glimelius, B., Norling, B., Westermark, B., and Wasteson, Å., 1978, Composition and distribution of glycosaminoglycans in cultures of human normal and malignant glial cells, Biochem. J. 172:443.

    PubMed  CAS  Google Scholar 

  • Glössl, J., Truppe, W., and Kresse, H., 1979, Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta, Biochem. J. 181:37.

    PubMed  Google Scholar 

  • Gniot-Szulzycka, J., and Donnelly, P. V., 1976, Arylsulphatase B (Maroteaux-Lamy factor): A part of the enzyme system responsible for sulphate release from mucopolysaccharide fragment, FEBS Lett. 65:63.

    PubMed  CAS  Google Scholar 

  • Grebner, E. E., Hall, C. W., and Neufeld, E. F., 1966a, Incorporation of d-xylose-14C into glycoprotein by particles from hen oviduct, Biochem. Biophys. Res. Commun. 22:672.

    PubMed  CAS  Google Scholar 

  • Grebner, E. E., Hall, C. W., and Neufeld, E. F., 1966b, Glycosylation of serine residues by a undine disphosphate-xylose:protein xylosyltransferase from mouse mastocytoma, Arch. Biochem. Biophys. 116:391.

    PubMed  CAS  Google Scholar 

  • Gregory, J. D., and Rodén, L., 1961, Isolation of keratosulfate from chondromucoprotein of bovine nasal septa, Biochem. Biophys. Res. Commun. 5:430.

    CAS  Google Scholar 

  • Gregory, J. D., Laurent, T. C., and Rodén, L., 1964, Enzymatic degradation of chondromucoprotein, J. Biol. Chem. 239:3312.

    PubMed  CAS  Google Scholar 

  • Greiling, H., Momburg, M., and Stuhlsatz, H. W., 1972, Isolation and substrate specificity of a PAPS: chondroitin 6-sulfotransferase from mouse liver, Scand. J. Clin. Lab. Invest. 29(Suppl. 123):37.

    Google Scholar 

  • Habuchi, H., Yamagata, T., Iwata, H., and Suzuki, S., 1973, The occurrence of a wide variety of dermatan sulfate-chondroitin sulfate copolymers in fibrous cartilage, J. Biol. Chem. 248:6019.

    PubMed  CAS  Google Scholar 

  • Hagopian, A., Bosmann, H. B., and Eylar, E. H., 1968, Glycoprotein biosynthesis: The localization of polypeptidyl: N-acetylgalactosaminyl, collagen:glucosyl, and glycoprotein:galactosyl transferases in HeLa cell membrane fractions, Arch. Biochem. Biophys. 128:387.

    PubMed  CAS  Google Scholar 

  • Hall, C. W., and Neufeld, E. F., 1973, α-l-Iduronidase activity in cultured skin fibroblasts and amniotic fluid cells, Arch. Biochem. Biophys. 158:817.

    PubMed  CAS  Google Scholar 

  • Hall, C. W., Cantz, M., and Neufeld, E. F., 1973, A β-glucuronidase deficiency mucopolysaccharidosis: Studies in cultured fibroblasts, Arch. Biochem. Biophys. 155:32.

    PubMed  CAS  Google Scholar 

  • Hall, C. W., Liebaers, I., Di Natale, P., and Neufeld, E. F., 1978, Enzymic diagnosis of the genetic mucopolysaccharide storage disorders, Methods Enzymol. 50:439.

    PubMed  CAS  Google Scholar 

  • Hamerman, D., Rojkind, M., and Sandson, J., 1966, Protein bound to hyaluronate: Chemical and immunological studies, Fed. Am. Soc. Exp. Biol. 25:1040.

    CAS  Google Scholar 

  • Handley, C. J., and Lowther, D. A., 1977, Extracellular matrix metabolism by chondrocytes. III. Modulation of proteoglycan synthesis by extracellular levels of proteoglycan in cartilage cells in culture, Biochim. Biophys. Acta 500:132.

    PubMed  CAS  Google Scholar 

  • Hardingham, T. E., 1979, The role of link-protein in the structure of cartilage proteoglycan aggregates, Biochem. J. 177:237.

    PubMed  CAS  Google Scholar 

  • Hardingham, T. E., and Muir, H., 1972, The specific interaction of hyaluronic acid with cartilage proteoglycan, Biochim. Biophys. Acta 279:401.

    PubMed  CAS  Google Scholar 

  • Hardingham, T. E., and Muir, H., 1974, Hyaluronic acid in cartilage and proteoglycan aggregation, Biochem. J. 139:565.

    PubMed  CAS  Google Scholar 

  • Hardingham, T. E., and Phelps, C. F., 1968, The tissue content and turnover rates of intermediates in the biosynthesis of glycosaminoglycans in young rat skin, Biochem. J. 108:9.

    PubMed  CAS  Google Scholar 

  • Hart, G. W., and Lennarz, W. J., 1978, Effects of tunicamyin on the biosynthesis of glycosaminoglycans by embryonic chick cornea, J. Biol. Chem. 253:5795.

    PubMed  CAS  Google Scholar 

  • Hascall, V. C., 1977, Interaction of cartilage proteoglycans with hyaluronic acid, J. Supramol. Struct. 7:101.

    PubMed  CAS  Google Scholar 

  • Hascall, V. C., and Heinegård, D., 1974, Aggregation of cartilage proteoglycans. I. The role of hyaluronic acid, J. Biol. Chem. 249:4232.

    PubMed  CAS  Google Scholar 

  • Hascall, V. C., and Sajdera, S. W., 1969, Proteinpolysaccharide complex from bovine nasal cartilage: The function of glycoprotein in the formation of aggregates, J. Biol. Chem. 244:2384.

    PubMed  CAS  Google Scholar 

  • Hascall, V. C., and Sajdera, S. W., 1970, Physical properties and polydispersity of proteoglycan from bovine nasal cartilage, J. Biol. Chem. 245:4920.

    PubMed  CAS  Google Scholar 

  • Haug, A., and Larsen, B., 1971, Biosynthesis of alginate. Part II. Polymannuronic acid C-5-epimerase from Azotobacter vinelandii (Lipman), Carbohydr. Res. 17:297.

    PubMed  CAS  Google Scholar 

  • Heidelberger, M., and Kendall, F. E., 1929, A crystalline aldobionic acid derived from gum arabic, J. Biol. Chem. 84:639.

    CAS  Google Scholar 

  • Heinegård, D., 1972, Extraction, fractionation and characterization of proteoglycans from bovine tracheal cartilage, Biochem. Biophys. Acta 285:181.

    PubMed  Google Scholar 

  • Helting, T., 1971, Biosynthesis of heparin: Solubilization, partial separation, and purification of uridine diphosphate-galactose: Acceptor galactosyltransferases from mouse mastocytoma, J. Biol. Chem. 246:815.

    PubMed  CAS  Google Scholar 

  • Helting, T., 1972, Biosynthesis of heparin: Solubilization and partial purification of uridine disphosphate glucuronic acid: Acceptor glucuronosyltransferase from mouse mastocytoma, J. Biol. Chem. 247:4327.

    PubMed  CAS  Google Scholar 

  • Helting, T., and Lindahl, U., 1971, Occurrence and biosynthesis of β-glucuronidic linkages in heparin, J. Biol. Chem. 246:5442.

    PubMed  CAS  Google Scholar 

  • Helting, T., and Lindahl, U., 1972, Biosynthesis of heparin. I. Transfer of N-acetylglucosamine and glucuronic acid to low-molecular weight heparin fragments, Acta Chem. Scand. 26:3515.

    PubMed  CAS  Google Scholar 

  • Helting, T., and Rodén, L., 1969a, Biosynthesis of chondroitin sulfate. I. Galactosyl transfer in the formation of the carbohydrate-protein linkage region, J. Biol. Chem. 244:2790.

    PubMed  CAS  Google Scholar 

  • Helting, T., and Rodén, L., 1969b, Biosynthesis of chondroitin sulfate. II. Glucuronosyl transfer in the formation of the carbohydrate-protein linkage region, J. Biol. Chem. 244:2799.

    PubMed  CAS  Google Scholar 

  • Helwig, J.-J., Farooqui, A. A., Bollack, C., and Mandel, P., 1977, Purification and some properties of arylsulphatases A and B from rabbit kidney cortex, Biochem. J. 165:127.

    PubMed  CAS  Google Scholar 

  • Hemming, F. W., 1974, Lipids in glycan biosynthesis, in: MTP International Review of Science, Ser. 1, Vol. 4, Biochemistry of Lipids (T. W. Goodwin, ed.), p. 39, University Park Press, Baltimore.

    Google Scholar 

  • Hestrin, S., Avineri-Shapiro, S., and Aschner, M., 1943, The enzymic production of levan, Biochem. J. 37:450.

    PubMed  CAS  Google Scholar 

  • Himeno, M., Hashiguchi, Y., and Kato, K., 1974, β-Glucuronidase of bovine liver. Purification, properties, carbohydrate composition, J. Biochem. 76:1243.

    PubMed  CAS  Google Scholar 

  • Himeno, M., Ohhara, H., Arakawa, Y., and Kato, K., 1975, β-Glucuronidase of rat preputial gland, J. Biochem. 77:427.

    PubMed  CAS  Google Scholar 

  • Himeno, M., Nishimura, Y., Tsuji, H., and Kato, K., 1976, Purification and characterization of microsomal and lysosomal β-glucuronidase from rat liver by use of immunoaffinity chromatography, Eur. J. Biochem. 70:349.

    CAS  Google Scholar 

  • Hirano, S., and Hoffman, P., 1962, The hexosaminidic linkage of hyaluronic acid, J. Org. Chem. 27:395.

    CAS  Google Scholar 

  • Hoffman, P., Linker A., and Meyer, K., 1956a, Uronic acid of chondroitin sulfate B, Science 124:1252.

    PubMed  CAS  Google Scholar 

  • Hoffman, P., Meyer, K., and Linker, A., 1956b, Transglycosylation during the mixed digestion of hyaluronic acid and chondroitin sulfate by testicular hyaluronidase, J. Biol. Chem. 219:653.

    PubMed  CAS  Google Scholar 

  • Hoffman, P., Linker A., and Meyer, K., 1957a, The acid mucopolysaccharides of connective tissues. II. Further experiments on chondroitin sulfate B, Arch. Biochem. Biophys. 69:435.

    PubMed  CAS  Google Scholar 

  • Hoffman, P., Linker, A., Sampson, P., Meyer, K., and Korn, E. D., 1957b, The degradation of hyaluronate, the chondroitin sulfates and heparin by bacterial enzymes (flavobac-terium), Biochim. Biophys. Acta 25:658.

    PubMed  CAS  Google Scholar 

  • Hoffman, P., Linker, A., Lippman, V., and Meyer, K., 1960, The structure of chondroitin sulfate B from studies with flavobacterium enzymes, J. Biol. Chem. 235:3066.

    PubMed  CAS  Google Scholar 

  • Höök, M., Lindahl, U., Bäckström, G., Malmstrom, A., and Fransson, L.-Å., 1974, Biosynthesis of heparin. III. Formation of iduronic acid residues, J. Biol. Chem. 249:3908.

    PubMed  Google Scholar 

  • Höök, M., Lindahl, U., Hallen, and Bäckström, G., 1975a, Biosynthesis of heparin: Studies on the microsomal sulfation process, J. Biol. Chem. 250:6065.

    PubMed  Google Scholar 

  • Höök, M., Wasteson, Å., and Oldberg, Å., 1975b, A heparan sulfate-degrading endoglycosidase from rat liver tissue, Biochem. Biophys. Res. Commun. 67:1422.

    PubMed  Google Scholar 

  • Höök, M., Pettersson, I., and Ögren, S., 1977, A heparin-degrading endoglycosidase from rat spleen, Thromb. Res. 10:857.

    PubMed  Google Scholar 

  • Hopwood, J., 1972, Cartilage proteoglycans: Biosynthesis and chemical structure, Ph.D. thesis, Monash University, Clayton, Australia.

    Google Scholar 

  • Hopwood, J. J., 1979, ∝-L-Iduronidase, β-d-glucuronidase, and 2-sulfo-l-iduronate 2-sulfatase: Preparation and characterization of radioactive substrates from heparin, Carbohydr. Res. 69:203.

    PubMed  CAS  Google Scholar 

  • Hopwood, J. J., and Robinson, H. C., 1974, The alkali-labile linkage between keratan sulphate and protein, Biochem. J. 141:57.

    PubMed  CAS  Google Scholar 

  • Horner, A. A., 1971, Macromolecular heparin from rat skin: Isolation, characterization, and depolymerization with ascorbate, J. Biol. Chem. 246:231.

    PubMed  CAS  Google Scholar 

  • Horner, A. A., 1972, Enzymic depolymerization of macromolecular heparin as a factor in control of lipoprotein lipase activity, Proc. Nat. Acad. Sci. U.S.A. 69:3469.

    CAS  Google Scholar 

  • Horowitz, M. I., 1977, Gastrointestinal glycoproteins, in: The Glycoconjugaies, Vol. II (M. I. Horowitz and W. Pigman, eds.), p. 189, Academic Press, New York.

    Google Scholar 

  • Horwitz, A. L., 1972, Cellular sites and enzymic mechanisms for synthesis of chondromucoprotein of cartilage, Ph.D. thesis, University of Chicago.

    Google Scholar 

  • Horwitz, A. L., and Dorfman, A., 1978, The enzymic defect in Morquio’s disease: The specificity of N-acetylhexosamine sulfatases, Biochem. Biophys. Res. Commun. 80:819.

    PubMed  CAS  Google Scholar 

  • Ishimoto, N., and Strominger, J. L., 1967, Uridine diphosphate as the sole uridine nucleotide product of hyaluronic acid synthetase in group A streptococci, Biochim. Biophys. Acta 148:296.

    PubMed  CAS  Google Scholar 

  • Jacobson, B., and Davidson, E. A., 1962, Biosynthesis of uronic acids by skin enzymes. II. Uridine diphosphate-d-glucuronic acid-5-epimerase, J. Biol. Chem. 237:638.

    PubMed  CAS  Google Scholar 

  • Jacobson, B., and Davidson, E. A., 1963, UDP-d-glucuronic acid-5-epimerase and UDP-N-acetylglucosamine-4-epimerase of rabbit skin, Biochim. Biophys. Acta 73:145.

    PubMed  CAS  Google Scholar 

  • Jacobsson, L, Bäckström, G., Höök, M., Lindahl, U., Feingold, D. S., Malmström, A., and Rodén, L., 1979, Biosynthesis of heparin: Assay and properties of the microsomal uronosyl C-5 epimerase, J. Biol. Chem. 254:2975.

    PubMed  CAS  Google Scholar 

  • Jansson, L., Höök, M., Wasteson, Å., and Lindahl, U., 1975, Biosynthesis of heparin. V. Solubilization and partial characterization of N-and O-sulphotransferases, Biochem. J. 149:49.

    PubMed  CAS  Google Scholar 

  • Jaques, L. B., 1940, Heparinase, J. Biol. Chem. 133:445.

    CAS  Google Scholar 

  • Johnson, A. H., and Baker, J. R., 1973, The enzymatic sulphation of heparin sulphate by hen’s uterus, Biochim. Biophys. Acta 320:341.

    PubMed  CAS  Google Scholar 

  • Kaplan, D., 1969, Classification of the mucopolysaccharidoses based on the pattern of mucopolysacchariduria, Am. J. Med. 47:721.

    PubMed  CAS  Google Scholar 

  • Reiser, H., Shulman, H. J., and Sandson, J. I., 1972, Immunochemistry of cartilage proteoglycan: Immunodiffusion and gel-electrophoretic studies, Biochem. J. 126:163.

    Google Scholar 

  • Kiely, M. L., McKnight, G. S., and Schimke, R. T., 1976, Studies on the attachment of carbohydrate to ovalbumin nascent chains in hen oviduct, J. Biol. Chem. 251:5490.

    PubMed  CAS  Google Scholar 

  • Kindler, A., Klein, U., and von Figura, K., 1977, Characterization of glycosaminoglycans stored in mucopolysaccharidosis. IIIA: Evidence for a generally occurring degradation of heparan sulfate by endoglycosidases, Hoppe-Seyler’s Z. Physiol. Chem. 358:1431.

    PubMed  CAS  Google Scholar 

  • Klein, U., and von Figura, K., 1976a, Demonstration of d-glucuronic acid as reducing terminal of intracellular heparan sulfates, FEBS Lett. 71:266.

    PubMed  CAS  Google Scholar 

  • Klein, U., and von Figura, K., 1976b, Partial purification and characterization of a heparan sulfate specific endoglucuronidase, Biochem. Biophys. Res. Commun. 73:569.

    PubMed  CAS  Google Scholar 

  • Klein, U., Kresse, H., and von Figura, K., 1976, Evidence for degradation of heparan sulfate by endoglycosidases: Glucosamine and hexuronic acid are reducing terminals of intracellular heparan sulfate from human skin fibroblasts, Biochem. Biophys. Res. Commun. 69:158.

    PubMed  CAS  Google Scholar 

  • Klein, U., Kresse, H., and von Figura, K., 1978, Sanfilippo syndrome type C: Deficiency of acetyl-CoA: ∝-glucosaminide N-acetyltransferase in skin fibroblasts, Proc. Natl. Acad. Sci. U.S.A. 75:5185.

    PubMed  CAS  Google Scholar 

  • Knecht, J., Cifonelli, J. A., and Dorfman, A., 1967, Structural studies on heparitin sulfate of normal and Hurler tissues, J. Biol. Chem. 242:4652.

    PubMed  CAS  Google Scholar 

  • Kornfeld, R., 1967, Studies on L-glutamine d-fructose 6-phosphate amido-transferase. I. Feedback inhibition by uridine diphosphate-N-acetylglucosamine, J. Biol. Chem. 242:3135.

    PubMed  CAS  Google Scholar 

  • Kornfeld, S., Kornfeld, R., Neufeld, E. F., and O’Brien, P. J., 1964, The feedback control of sugar nucleotide biosynthesis in liver, Proc. Natl. Acad. Sci. U.S.A. 52:371.

    PubMed  CAS  Google Scholar 

  • Kraemer, P. M., 1971, Heparan sulfates of cultured cells. I. Membrane-associated and cell-sap species in Chinese hamster cells. Biochemistry 10:1437.

    PubMed  CAS  Google Scholar 

  • Kraemer, P. M., 1977, Heparin releases heparan sulfate from the cell surface, Biochem. Biophys. Res. Commun. 78:1334.

    PubMed  CAS  Google Scholar 

  • Kresse, H., 1973, Mucopolysaccharidosis III A (Sanfilippo A disease): Deficiency of a heparin sulfamidase in skin fibroblasts and leucocytes, Biochem. Biophys. Res. Commun. 54:1111.

    PubMed  CAS  Google Scholar 

  • Kresse, H., and Neufeld, E. F., 1972, The Sanfilippo A corrective factor: Purification and mode of action, J. Biol. Chem. 247:2164.

    PubMed  CAS  Google Scholar 

  • Kresse, H., Wiesmann, U., Cantz, M., Hall, C. W., and Neufeld, E. F., 1971, Biochemical heterogeneity of the Sanfilippo syndrome: Preliminary characterization of two deficient factors, Biochem. Biophys. Res. Commun. 42:892.

    PubMed  CAS  Google Scholar 

  • Larsen, B., and Haug, A., 1971, Biosynthesis of alginate. Part III. Tritium incorporation wit polymannuronic acid 5-epimerase from Azotobacter vinelandii, Carbohydr. Res. 20:225.

    CAS  Google Scholar 

  • Leaback, D. H., 1970, The metabolic hydrolysis of hexosaminide linkages, in: Metabolic Conjugation and Metabolic Hydrolysis, Vol. 2 (W. H. Fishman, ed.), p. 443, Academic Press, New York.

    Google Scholar 

  • Leloir, L. F., 1972, Biosynthesis of polysaccharides seen from Buenos Aires, in: Biochemistry of the Glycosidic Linkage (R. Piras and H. G. Pontis, eds.), p. 1, Academic Press, New York.

    Google Scholar 

  • Lemaire, A., Picard, J., and Gardais, A., 1967, Le catabolisme de l’héparine: Étude in vivode la désulfatation de l’héparine chez le rat, C. R. Acad. Sci. Paris 264:949.

    CAS  Google Scholar 

  • Lennarz, W. J., 1975, Lipid linked sugars in glycoprotein synthesis, Science 188:986.

    PubMed  CAS  Google Scholar 

  • Levvy, G. A., and Conchie, J., 1966, β-Glucuronidase and the hydrolysis of gluronides, in: Glucuronic Acid, Free and Combined (G. J. Dutton, ed.), p. 301, Academic Press, New York.

    Google Scholar 

  • Liebaers, I., and Neufeld, E. F., 1976, Iduronate sulfatase activity in serum, lymphocytes, and fibroblasts —Simplified diagnosis of the Hunter syndrome, Pediatr. Res. 10:733.

    PubMed  CAS  Google Scholar 

  • Liebaers, I., Di Natale, P., and Neufeld, E. F., 1977, Iduronate sulfatase in amniotic fluid: An aid in the prenatal diagnosis of the Hunter syndrome, J. Pediatr. 90:423.

    PubMed  CAS  Google Scholar 

  • Lim, T. W., Leder, I. G., Bach, G., and Neufeld, E. F., 1974, An assay for iduronate sulfatase (Hunter corrective factor), Carbohydr. Res. 37:103.

    PubMed  CAS  Google Scholar 

  • Lindahl, U., 1966a, The structures of xylosylserine and galactosylxylosylserine from heparin, Biochim. Biophys. Acta 130:361.

    PubMed  CAS  Google Scholar 

  • Lindahl, U., 1966b, Further characterization of the heparin-protein linkage region, Biochim. Biophys. Acta 130:368.

    PubMed  CAS  Google Scholar 

  • Lindahl, U., 1976, Structure and biosynthesis of l-iduronic acid-containing glycosaminoglycans, in: MTP International Review of Science: Organic Chemistry Series TwoCarbohydrate Chemistry (G. O. Aspinall, ed.), Vol. 7, p. 283, Butterworths, London.

    Google Scholar 

  • Lindahl, U., and Axelsson, O., 1971, Identification of iduronic acid as the major sulfated uronic acid of heparin, J. Biol. Chem. 246:74.

    PubMed  CAS  Google Scholar 

  • Lindahl, U., and Höök, M., 1978, Glycosaminoglycans and their binding to biological macromolecules, Annu. Rev. Biochem. 47:385.

    PubMed  CAS  Google Scholar 

  • Lindahl, U., and Rodén, L., 1964, The linkage of heparin to protein, Biochem. Biophys. Res. Commun. 17:254.

    PubMed  CAS  Google Scholar 

  • Lindahl, U., and Rodén, L., 1965, The role of galactose and xylose in the linkage of heparin to protein, J. Biol. Chem. 240:2821.

    PubMed  CAS  Google Scholar 

  • Lindahl, U., and Rodén, L., 1972, Carbohydrate-peptide linkages in proteoglycans of animal, plant and bacterial origin, in: Glycoproteins (A. Gottschalk, ed.), p. 491, Elsevier, Amsterdam.

    Google Scholar 

  • Lindahl, U., Cifonelli, J. A., Lindahl, B., and Rodén, L., 1965, The role of serine in the linkage of heparin to protein, J. Biol. Chem. 240:2817.

    PubMed  CAS  Google Scholar 

  • Lindahl, U., Bäckström, G., Malmström, A., and Fransson, L.-Å., 1972, Biosynthesis of l-iduronic acid in heparin: Epimerization of D-glucuronic acid on the polymer level, Biochem. Biophys. Res. Commun. 46:985.

    PubMed  CAS  Google Scholar 

  • Lindahl, U., Bäckström, G., Jansson, L., and Hallén, A., 1973, Biosynthesis of heparin. II. Formation of sulfamino groups, J. Biol. Chem. 248:7234.

    PubMed  CAS  Google Scholar 

  • Lindahl, U., Jacobsson, I., Höök, M., Bäckström, G., and Feingold, D. S., 1976, Biosynthesis of heparin. VI. Loss of C-5 hydrogen during conversion of d-glucuronic to l-iduronic acid residues, Biochem. Biophys. Res. Commun. 70:492.

    PubMed  CAS  Google Scholar 

  • Lindahl, U., Höök, M., Bäckström, G., Jacobsson, L, Riesenfeld, J., Malmström, A., Rodén, L., and Feingold, D. S., 1977, Structure and biosynthesis of heparin-like polysaccharides, Fed. Proc. Fed. Am. Soc. Exp. Biol. 36:19.

    CAS  Google Scholar 

  • Linker, A., Meyer, K., and Weissmann, B., 1955, Enzymatic formation of monosaccharides from hyaluronate, J. Biol. Chem. 213:237.

    PubMed  CAS  Google Scholar 

  • Linker, A., Meyer, K., and Hoffman, P., 1956, The production of unsaturated uronides by bacterial hyaluronidases, J. Biol. Chem. 219:13.

    PubMed  CAS  Google Scholar 

  • Lloyd, A. G., Embery, G., Wusterman, F. S., and Dodgson, K. S., 1966, The metabolic fate of 35S-labeled heparin and related compounds, Biochem. J. 98:33.

    Google Scholar 

  • Lloyd, A. G., Fowler, L. J., Embery, G., and Lau, B. A., 1968, Degradation of [35S]heparin by mammalian and bacterial sulphamidases, Biochem. J. 110:54P.

    Google Scholar 

  • Malawista, I., and Schubert, M., 1958, Chondromucoprotein: New extraction method and alkaline degradation, J. Biol. Chem. 230:535.

    PubMed  CAS  Google Scholar 

  • Malmström, A., and Fransson, L.-Å., 1971a, Structure of pig skin dermatan sulfate. 2. Demonstration of sulfated iduronic acid residues, Eur. J. Biochem. 18:431.

    PubMed  Google Scholar 

  • Malmström, A., and Fransson, L.-Å., 1971, Studies on the biosynthesis of dermatan sulfate, FEBS Lett. 16:105.

    PubMed  Google Scholar 

  • Malmström, A., Carlstedt, L, Åberg, L., and Fransson, L.-Å., 1975a, The copolymeric structure of dermatan sulphate from cultured human fibroblasts, Biochem. J. 151:477.

    Google Scholar 

  • Malmström, A., Fransson, L.-Â., Höök, M., and Lindahl, U., 1975b, Biosynthesis of dermatan sulfate. I. Formation of L-iduronic acid residues, J. Biol. Chem. 250:3419.

    PubMed  Google Scholar 

  • Malmström, A., Bäckström, G., Feingold, D. S., Höök, M., Jacobsson, I., Lindahl, U., Riesenfeld, J., and Rodën, L., 1976, Biosynthesis of heparin: Purification of the C-5-uronosyl epimerase, Fed. Proc. Fed. Am. Soc. Exp. Biol. 35:1374 (abstract).

    Google Scholar 

  • Malmström, A., Rodén, L., Feingold, D., Jacobsson, I., Bäckström, G., Höök, M., and Lindahl, U., 1980, Biosynthesis of heparin. Partial purification of the uronosyl C-5 epimerase, J. Biol. Chem., in press.

    Google Scholar 

  • Marbet, R., and Winterstein, A., 1951, β-Heparin, ein neuer, blutgerinnungshemmender Mucoitinschwefelsäureester, Helv. Chim. Acta 34:2311.

    CAS  Google Scholar 

  • Markovitz, A., and Dorfman, A., 1962, Synthesis of capsular polysaccharide (hyaluronic acid) by protoplast membrane preparations of group A streptococcus, J. Biol. Chem. 237:273.

    PubMed  CAS  Google Scholar 

  • Markovitz, A., Cifonelli, J. A., and Dorfman, A., 1959, The biosynthesis of hyaluronic acid by group A streptococcus. VI. Biosynthesis from uridine nucleotides in cell-free extracts, J. Biol. Chem. 234:2343.

    PubMed  CAS  Google Scholar 

  • Matalon, R., and Dorfman, A., 1968, The structure of acid mucopolysaccharides produced by Hurler fibroblasts in tissue culture, Proc. Natl. Acad. Sci. U.S.A. 60:179.

    PubMed  CAS  Google Scholar 

  • Matalon, R., and Dorfman, A., 1972, Hurler’s syndrome, an ∝-L-iduronidase deficiency, Biochem. Biophys. Res. Commun. 47:959.

    PubMed  CAS  Google Scholar 

  • Matalon, R., and Dorfman, A., 1973, Sanfilippo A syndrome: A sulfamidase deficiency, Pediatr. Res. 7:384.

    Google Scholar 

  • Matalon, R., and Dorfman, A., 1974, Sanfilippo A syndrome: Sulfaminidase deficiency in cultured skin fibroblasts and liver, J. Clin. Invest. 54:907.

    PubMed  CAS  Google Scholar 

  • Matalon, R., Cifonelli, J. A., and Dorfman, A., 1971, l-Iduronidase in cultured human fibroblasts and liver, Biochem. Biophys. Res. Commun. 42:340.

    PubMed  CAS  Google Scholar 

  • Matalon, R., Arbogast, B., and Dorfman, A., 1974a, Deficiency of chondroitin sulfate N-acetylgalactosamine 4-sulfate sulfatase in Maroteaux-Lamy syndrome, Biochem. Biophys. Res. Commun. 61:1450.

    PubMed  CAS  Google Scholar 

  • Matalon, R., Arbogast, B., Justice, P., Brandt, I. K., and Dorfman, A., 1974b, Morquio’s syndrome: Deficiency of a chondroitin sulfate N-acetylhexosamine sulfate sulfatase, Biochem. Biophys. Res. Commun. 61:759.

    PubMed  CAS  Google Scholar 

  • Mathews, M. B., 1958, Isomeric chondroitin sulphates, Nature (London) 181:421.

    CAS  Google Scholar 

  • Mathews, M. B., 1975, Connective Tissue, Springer-Verlag, New York.

    Google Scholar 

  • Mathews, M. B., and Dorfman, A., 1955, Inhibition of hyaluronidase, Physiol. Rev. 35:381.

    PubMed  CAS  Google Scholar 

  • Mathews, M. B., and Lozaityte, I., 1958, Sodium chondroitin sulfate-protein complexes of cartilage. I. Molecular weight and shape, Arch. Biochem. Biophys. 74:158.

    PubMed  CAS  Google Scholar 

  • McKusick, V. A., Neufeld, E. F., and Kelly, T. E., 1978, The mucopolysaccharide storage diseases, in: The Metabolic Basis of Inherited Disease,4th ed. (J. B. Stanbury, J. B. Wyngaarden, and D. S. Fredrickson, eds.), p. 1282, McGraw-Hill, New York.

    Google Scholar 

  • Meyer, K., 1970, Reflections on “mucopolysaccharides” and their protein complexes, in: Chemistry and Molecular Biology of the Intercellular Matrix (E. A. Balazs, ed.), p. 5, Academic Press, New York.

    Google Scholar 

  • Meyer, K., and Chaffee, E., 1941, The mucopolysaccharides of skin, J. Biol. Chem. 138:491.

    CAS  Google Scholar 

  • Meyer, K., Davidson, E., Linker, A., and Hoffman, P., 1956, The acid mucopolysaccharides of connective tissue, Biochim. Biophys. Acta 21:506.

    PubMed  CAS  Google Scholar 

  • Muir, H., and Hardingham, T. E., 1975, Structure of proteoglycans, in: Biochemistry of Carbohydrates, MTP International Review of Science, Biochemistry Series One, Vol. 5 (W. J. Whelan, ed.), p. 153, Butterworths, London.

    Google Scholar 

  • Neufeld, E. F., and Fratantoni, J. C., 1970, Inborn errors of mucopolysaccharide metabolism, Science 169:141.

    PubMed  CAS  Google Scholar 

  • Neufield, E. F., and Hall, C. W., 1965, Inhibition of UDP-d-glucose dehydrogenase by UDP-d-xylose: A possible regulatory mechanism, Biochem. Biophys. Res. Commun. 19:456.

    Google Scholar 

  • Neufeld, E. F., Lim, T. W., and Shapiro, L. J., 1975, Inherited disorders of lysosomal metabolism, Annu. Rev. Biochem. 44:357.

    PubMed  CAS  Google Scholar 

  • Norden, A. G. W., Tennant, L. L., and O’Brien, J. S., 1974, Gm1 ganglioside β-galactosidase A: Purification and studies of the enzyme from human liver, J. Biol. Chem. 249:7969.

    PubMed  CAS  Google Scholar 

  • O’Brien, J. S., 1972, Sanfilippo syndrome: Profound deficiency of alpha-acetylglucosaminidase activity in organs and skin fibroblasts from type-B patients, Proc. Natl. Acad. Sci. U.S.A. 69:1720.

    PubMed  Google Scholar 

  • O’Brien, J. S., 1978, The gangliosidoses, in: The Metabolic Basis of Inherited Disease (J. B. Stanbury, J. B. Wyngaarden, and D. S. Fredrickson, eds.), p. 841, McGraw-Hill, New York.

    Google Scholar 

  • O’Brien, J. S., Cantz, M., and Spranger, J., 1974, Maroteaux-Lamy disease (mucopoly-saccharidosis VI), subtype A: Deficiency of a N-acetylgalactosamine-4-sulfatase, Biochem. Biophys. Res. Commun. 60:1170.

    PubMed  Google Scholar 

  • O’Brien, J. S., Gugler, E., Giedion, A., Wiessmann, U., Herschkowitz, N., Meier, C., and Leroy, J., 1976, Spondyloepiphyseal dysplasia, corneal clouding, normal intelligence and acid β-galactosidase deficiency, Clin. Genet. 9:495.

    PubMed  Google Scholar 

  • Öbrink, B., 1972, Isolation and partial characterization of a dermatan sulphate-proteoglycan from pig skin, Biochim. Biophys. Acta 264:354.

    PubMed  Google Scholar 

  • Öckerman, P. A., 1968, Identity of β-glucosidase, β-xylosidase and one of the β-galactos-idase activities in human liver when assayed with 4-methylumbelliferyl-β-d-glycosides: Studies in cases of Gaucher’s disease, Biochim. Biophys. Acta 165:59.

    PubMed  Google Scholar 

  • Oegema, T. R., Hascall, V. C., and Dziewiatkowski, D. D., 1975, Isolation and characterization of proteoglycans from the Swarm rat chondrosarcoma, J. Biol. Chem. 250:6151.

    PubMed  CAS  Google Scholar 

  • Oegema, T. R., Brown, M., and Dziewiatkowski, D. D., 1977, The link protein in proteoglycan aggregates from the Swarm rat chondrosarcoma, J. Biol. Chem. 252:6470.

    PubMed  CAS  Google Scholar 

  • Ögren, S., and Lindahl, U., 1975, Cleavage of macromolecular heparin by enzyme and mouse mastocytoma, J. Biol. Chem. 250:2690.

    PubMed  Google Scholar 

  • Okayama, M., and Lowther, D. A., 1973, Effects of β-xylosides on the synthesis of chondroitin sulfate-protein complexes in cartilage slices, Proc. Aust. Biochem. Soc. 6:75.

    Google Scholar 

  • Okayama, M., Kimata, K., and Suzuki, S., 1973, The influence of p-nitrophenyl β-d-xyloside on the synthesis of proteochondroitin sulfate by slices of embryonic chick cartilage, J. Biochem. 74:1069.

    PubMed  CAS  Google Scholar 

  • Oldberg, Å., Höök, M., Öbrink, B., Pertoft, H., and Rubin, K., 1977, Structure and metabolism of rat liver heparan sulphate, Biochem. J. 164:75.

    PubMed  CAS  Google Scholar 

  • Oldberg, Å., Kjellén, L., and Höök, M., 1979, Cell-surface heparan sulfate: Isolation and characterization of a proteoglycan from rat liver membranes, J. Biol. Chem. 254:8505.

    PubMed  CAS  Google Scholar 

  • Orkin, R. W., Jackson, G., and Toole, B. P., 1977, Hyaluronidase activity in cultured chick embryo skin fibroblasts, Biochem. Biophys. Res. Commun. 77:132.

    PubMed  CAS  Google Scholar 

  • Patel, V., 1978, Degradation of glycoproteins, in: The Glycoconjugates, Vol. II (M. I. Horowitz and W. Pigman, eds.), p. 185, Academic Press, New York.

    Google Scholar 

  • Patel, V., and Tappel, A. L., 1969a, β-Glucosidase and β-xylosidase of rat kidney, Biochim. Biophys. Acta 191:653.

    PubMed  CAS  Google Scholar 

  • Patel, V., and Tappel, A. L., 1969b, Identity of β-glucosidase and β-xylosidase activities in rat liver lysosomes, Biochim. Biophys. Acta 191:86.

    PubMed  CAS  Google Scholar 

  • Perlman, R. L., Telser, A., and Dorfman, A., 1964, The biosynthesis of chondroitin sulfate by a cell-free preparation, J. Biol. Chem. 239:3623.

    PubMed  CAS  Google Scholar 

  • Platt, D., and Dorn, M., 1968, Nachweis, Reinigung und Eigenschaften der Glycosaminog-lycano-hydrolasen im menschlichen Hyalinen Knorpel, Clin. Chim. Acta 21:333.

    PubMed  CAS  Google Scholar 

  • Preston, B. N., 1968, Physical characterization of dermatan sulphate-protein, Arch. Biochem. Biophys. 126:974.

    PubMed  CAS  Google Scholar 

  • Rapport, M. M., Weissmann, B., Linker, A., and Meyer, K., 1951, Isolation of a crystalline disaccharide, hyalobiuronic acid, from hyaluronic acid, Nature (London) 168:996.

    CAS  Google Scholar 

  • Revell, P. A., and Muir, H., 1972, The excretion and degradation of chondroitin 4-sulphate administered to guinea pigs as free chondroitin sulphate and as proteoglycan, Biochem. J. 130:597.

    PubMed  CAS  Google Scholar 

  • Ringertz, N. R., 1963, Polysaccharides of neoplastic mast cells, Ann. N.Y. Acad. Sci. 103:209.

    PubMed  CAS  Google Scholar 

  • Robbins, P. W., Bray, D., Danhert, M., and Wright, A., 1967, Direction of chain growth in polysaccharide synthesis, Science 158:1536.

    PubMed  CAS  Google Scholar 

  • Robinson, D., and Abrahams, H. E., 1967, β-d-Xylosidase in pig kidney, Biochim. Biophys. Acta 132:212.

    PubMed  CAS  Google Scholar 

  • Robinson, H. C., Telser, A., and Dorfman, A., 1966, Studies on the biosynthesis of the linkage region of chondroitin-sulfate protein complex, Proc. Natl. Acad. Sci. U.S.A. 56:1859.

    PubMed  CAS  Google Scholar 

  • Robinson, H. C., Brett, M. J., Tralaggan, P. J., Lowther, D. A., and Okayama, M., 1975, The effect of d-xylose, β-d-xylosides and β-d-galactosides on chondroitin sulphate biosynthesis in embryonic chicken cartilage, Biochem. J. 148:25.

    PubMed  CAS  Google Scholar 

  • Robinson, H. C., Homer, A. A., Höök, M., Ögren, S., and Lindahl, U., 1978, A proteoglycan form of heparin and its degradation to single-chain molecules, J. Biol. Chem. 253:6687.

    PubMed  CAS  Google Scholar 

  • Roblin, R., Albert, S. O., Gelb, N. A., and Black, P. H., 1975, Cell surface changes correlated with density-dependent growth inhibition, glycosaminoglycan metabolism in 3T3, SV3T3, and Con A selected revenant cells, Biochemistry 14:347.

    PubMed  CAS  Google Scholar 

  • Rodén, L., 1970, Biosynthesis of acidic glycosaminoglycans (mucopolysaccharides), in: Metabolic Conjugation and Metabolic Hydrolysis, Vol. 2 (W. H. Fishman, ed.), p. 345, Academic Press, New York.

    Google Scholar 

  • Rodén, L., and Armand, G., 1966, Structure of the chondroitin-4-sulfate-protein linkage region: Isolation and characterization of the disaccharide 3-0-β-d-glucuronosyl-d-gal-actose, J. Biol. Chem. 241:65.

    PubMed  Google Scholar 

  • Rodén, L., and Horowitz, M. I., 1978, Proteoglycans and structural glycoproteins, in: The Glycoconjugates, Vol. II (M. I. Horowitz and W. Pigman, eds.), p. 3, Academic Press, New York.

    Google Scholar 

  • Rodén, L., and Schwartz, N. B., 1975. Biosynthesis of connective tissue proteoglycans, in: Biochemistry of Carbohydrates, MTP International Review of Science, Biochemistry Series One, Vol. 5 (W. J. Whelan, ed.), p. 95, Butterworths, London.

    Google Scholar 

  • Rome, L. H., Garvin, A. J., and Neufeld, E. F., 1978, Human kidney ∝-L-iduronidase: Purification and characterization, Arch. Biochem. Biophys. 189:344.

    PubMed  CAS  Google Scholar 

  • Roseman, S., and Dorfman, A., 1951, ∝-Glucosaminidase, J. Biol. Chem. 191:607.

    PubMed  CAS  Google Scholar 

  • Rosenberg, L., Hellmann, W., and Kleinschmidt, A. K., 1975, Electron microscope studies of proteoglycan aggregates from bovine articular cartilage, J. Biol. Chem. 250:1877.

    PubMed  CAS  Google Scholar 

  • Roy, A. B., 1953, The sulphatase of ox liver. I. The complex nature of the enzyme, Biochem. J. 53:12.

    PubMed  CAS  Google Scholar 

  • Roy, A. B., 1958, Comparative studies on the liver sulphatases, Biochem. J. 68:519.

    PubMed  CAS  Google Scholar 

  • Roy, A. B., 1976, Sulphatases, lysosomes and disease, Aust. J. Exp. Biol. Med. 54:111.

    CAS  Google Scholar 

  • Roy, A. B., and Trudinger, P. A., 1970, The Biochemistry of Inorganic Compounds of Sulphur, p. 133, Cambridge, New York.

    Google Scholar 

  • Sajdera, S. W., and Hascall, V. C., 1969, Proteinpolysaccharide complex from bovine nasal cartilage: A comparison of low and high shear extraction procedures, J. Biol. Chem. 244:77.

    PubMed  CAS  Google Scholar 

  • Sandson, J., and Hamerman, D., 1962, Isolation of hyaluronate protein from human synovial fluid, J. Clin. Invest. 41:1817.

    PubMed  CAS  Google Scholar 

  • Schachter, H., 1978, Glycoprotein biosynthesis, in: The Glycoconjugates, Vol. II (M. I. Horowitz and W. Pigman, eds.), p. 87, Academic Press, New York.

    Google Scholar 

  • Schachter, H., and Rodén, L., 1973, The biosynthesis of animal glyco proteins, in: Metabolic Conjugation and Metabolic Hydrolysis, Vol. 3 (W. H. Fishman, ed.), p. 1, Academic Press, New York.

    Google Scholar 

  • Scher, I., and Hamerman, D., 1972, Isolation of human synovial-fluid hyaluronate by density-gradient ultracentrifugation and evaluation of its protein content, Biochem. J. 126:1073.

    PubMed  CAS  Google Scholar 

  • Schiller, S., and Dorfman, A., 1957, The metabolism of mucopolysaccharides in animals. IV. The influence of insulin, J. Biol. Chem. 227:625.

    PubMed  CAS  Google Scholar 

  • Schubert, M., and Hamerman, D., 1968, A Primer on Connective Tissue Biochemistry, Lea and Febiger, Philadephia.

    Google Scholar 

  • Schwartz, N. B., 1975, Biosynthesis of chondroitin sulfate: Immunoprecipitation of interaction xylosyltransferase and galactosyltransferase, FEBS Lett. 49:342.

    PubMed  CAS  Google Scholar 

  • Schwartz, N. B., 1976a, Biosynthesis of chondroitin sulfate: Role of phospholipids in the activity of UDP-d-galactose: d-xylose galactosyltransferase, J. Biol. Chem. 251:285.

    PubMed  CAS  Google Scholar 

  • Schwartz, N. B., 1976b, Chondroitin sulfate glycosyltransferases in cultured chondrocytes: Turnover, oscillatory change during growth, and suppression by 5-bromodeoxyuridine, J. Biol. Chem. 251:3346.

    PubMed  CAS  Google Scholar 

  • Schwartz, N. B., 1977, Regulation of chondroitin sulfate synthesis: Effect of β-xylosides on synthesis of chondroitin sulfate proteoglycan, chondroitin sulfate chains, and core protein, J. Biol. Chem. 252:6316.

    PubMed  CAS  Google Scholar 

  • Schwartz, N. B., and Dorfman, A., 1975, Purification of rat liver chondrosarcoma xylosyltransferase, Arch. Biochem. Biophys. 171:136.

    PubMed  CAS  Google Scholar 

  • Schwartz, N. B., and Rodén, L., 1974, Biosynthesis of chondroitin sulfate: Purification of UDP-d-xylose: core protein β-d-xylosyltransferase by affinity chromgraphy, Carbohyd. Res. 37:167.

    CAS  Google Scholar 

  • Schwartz, N. B., and Rodén, L., 1975, Biosynthesis of chondroitin sulfate: Solubilization of chondroitin sulfate glycosyltransferases and partial purification of uridine diphosphate d-galactose: d-xylose galactosyltransferase, J. Biol. Chem. 250:5200.

    PubMed  CAS  Google Scholar 

  • Schwartz, N. B., Galligani, L., Ho, P.-L., and Dorfman, A., 1974a, Stimulation of synthesis of free chondroitin sulfate chains by β-d-xylosides in cultured cells, Proc. Natl. Acad. Sci. U.S.A. 71: 4047.

    PubMed  CAS  Google Scholar 

  • Schwartz, N. B., Rodén, L., and Dorfman, A., 1974b, Biosynthesis of chondroitin sulfate: Interaction between xylosyltransferase and galactosyltransferase, Biochem. Biophys. Res. Commun. 56:717.

    PubMed  CAS  Google Scholar 

  • Seno, N., Meyer, K., Anderson, B., Hoffman, P., 1965, Variations in keratosulfates, J. Biol. Chem. 240:1005.

    PubMed  CAS  Google Scholar 

  • Seno, N., Anno, K., Yaegashi, Y., and Okuyama, T., 1975, Microheterogeneity of chondroitin sulfates from various cartilages, Connect. Tissue Res. 3:87.

    PubMed  CAS  Google Scholar 

  • Shapiro, L. J., Hall, C. W., Leder, I. G., and Neufeld, E. F., 1976, The relationship of ∝-l-iduronidase and Hurler corrective factor, Arch. Biochem. Biophys. 172:156.

    PubMed  CAS  Google Scholar 

  • Shatton, J., and Schubert, M., 1954, Isolation of a mucoprotein from cartilage, J. Biol. Chem. 211:565.

    PubMed  CAS  Google Scholar 

  • Sheehan, J. K., Nieduszynski, I. A., Phelps, C. F., Muir, H., and Hardingham, T. E., 1978, Self-association of proteoglycan subunits from pig laryngeal cartilage, Biochem. J. 171:109.

    PubMed  CAS  Google Scholar 

  • Siewert, G., and Strominger, J. L., 1967, Bacitracin: An inhibitor of the dephosphorylation of lipid pyrophosphate, an intermediate in biosynthesis of the peptidoglycan of bacterial cells walls, Proc. Natl. Acad. Sci. U.S.A. 57:767.

    PubMed  CAS  Google Scholar 

  • Silbert, J. E., 1963, Incorporation of 14C and 3H from nucleotide sugars into a polysaccharide in the presence of a cell-free preparation from mouse mast cell tumors, J. Biol. Chem. 238:3542.

    PubMed  CAS  Google Scholar 

  • Silbert, J. E., 1964, Incorporation of 14C and 3H from labeled nucleotide sugars into a polysaccharide in the presence of a cell-free preparation from cartilage, J. Biol. Chem. 239:1310.

    PubMed  CAS  Google Scholar 

  • Silbert, J. E., 1967a, Incorporation of 35SO4 into endogenous heparin by a microsomal fraction from mast cell tumors, J. Biol. Chem. 242:2301.

    PubMed  CAS  Google Scholar 

  • Silbert, J. E., 1967b, Biosynthesis of heparin. III. Formation of a sulfated glycosaminoglycan with a microsomal preparation from mast cell tumors, J. Biol. Chem. 242:5146.

    PubMed  CAS  Google Scholar 

  • Silbert, J. E., 1967c, Biosynthesis of heparin. IV. N-deacetylation of a precursor glycosaminoglycan, J. Biol. Chem. 242:5153.

    PubMed  CAS  Google Scholar 

  • Singh, J., Di Ferrante, N., Niebes, P., and Tavella, D., 1976, N-acetylgalactosamine-6-sulfate sulfatase in man: Absence of the enzyme in Morquio disease, J. Clin. Invest. 57:1036.

    PubMed  CAS  Google Scholar 

  • Sjöberg, I., 1978, Structure and metabolism of glycosaminoglycans in human fibroblasts, M.D. thesis, University of Lund, Sweden.

    Google Scholar 

  • Sjöberg, I., Fransson, L.-Å., Matalon, R., and Dorfman, A., 1973, Hunter’s syndrome: A deficiency of L-idurono-sulfate sulfatase, Biochem. Biophys. Res. Commun. 54:1125.

    PubMed  Google Scholar 

  • Sly, W. S., Quinton, B. A., McAlister, W. H., and Rimoin, D. L., 1973, Beta glucuronidase deficiency: Report of clinical, radiologie, and biochemical features of a new mucopolysaccharidosis, J. Pediatr. 82:249.

    PubMed  CAS  Google Scholar 

  • Smith, H., and Gallop, R. C., 1953, The “acid polysaccharides” of hog gastric mucosa, Biochem. J. 53:666.

    PubMed  CAS  Google Scholar 

  • Spiro, M. J., 1977, Presence of a glucuronic acid-containing carbohydrate unit in human thyroglobulin, J. Biol. Chem. 252:5424.

    PubMed  CAS  Google Scholar 

  • Spranger, J. W., 1977, Beta galactosidase and the Morquio syndrome, Am. J. Med. Gen. 1:207.

    CAS  Google Scholar 

  • Srivastava, R. M., Hudson, N., Seymour, F. R., and Weissmann, B., 1978, Preparation of (aryl ∝-L-idopyranosid)uronic acids, Carbohydr. Res. 60:315.

    CAS  Google Scholar 

  • Stern, E. L., Lindahl, B., and Rodén, L., 1971, The linkage of dermatan sulfate to protein. I. Monosaccharide sequence of the linkage region, J. Biol. Chem. 246:5707.

    PubMed  CAS  Google Scholar 

  • Stevens, R. L., Fluharty, A. L., Killgrove, A. R., and Kihara, H., 1977, Arylsulfatases of human tissue: Studies on a form of arylsulfatase B found predominantly in brain, Biochim. Biophys. Acta 481:549.

    PubMed  CAS  Google Scholar 

  • Stoffyn, P. J., and Jeanloz, R. W., 1960, The identification of the uronic acid component of dermatan sulfate (β-heparin, chondroitin sulfate B), J. Biol. Chem. 235:2507.

    PubMed  CAS  Google Scholar 

  • Stoolmiller, A. C., and Dorfman, A., 1969a, The biosynthesis of hyaluronic acid by Streptococcus, J. Biol. Chem. 244: 236.

    CAS  Google Scholar 

  • Stoolmiller, A. C., and Dorfman, A., 1969b, The metabolism of glycosaminoglycans, in: Comprehensive Biochemistry, Vol. 17 (M. Florkin and E. H. Stotz, eds.), p. 241, Elsevier, Amsterdam.

    Google Scholar 

  • Stoolmiller, A. C., Horwitz, A. L., and Dorfman, A., 1972, Biosynthesis of the chondroitin sulfate proteoglycan: Purification and properties of xylosyltransferase, J. Biol. Chem. 247:3525.

    PubMed  CAS  Google Scholar 

  • Stumpf, D. A., Austin, J. H., Crocker, A. C., and LaFrance, M., 1973, Mucopolysac-charidosis type VI (Maroteaux-Lamy syndrome). I. Sulfatase B deficiency in tissues, Am. J. Dis. Child. 126:747.

    PubMed  CAS  Google Scholar 

  • Suzuki, S., 1960, Isolation of novel disaccharides from chondroitin sulfates, J. Biol. Chem. 235:3580.

    CAS  Google Scholar 

  • Suzuki, S., and Strominger, J. L., 1960, Enzymatic sulfation of mucopolysaccharides in hen oviduct. II. Mechanism of the reaction studied with oligosaccharides and mono-saccharides as acceptors, J. Biol. Chem. 235:267.

    PubMed  CAS  Google Scholar 

  • Telser, A., Robinson, H. C., and Dorfman, A., 1966, The biosynthesis of chondroitin sulfate, Arch. Biochem. Biophys. 116: 458.

    PubMed  CAS  Google Scholar 

  • Thomas, L., 1956, Reversible collapse of rabbit ears after intravenous papain, and prevention of recovery by cortisone, J. Exp. Med. 104:245.

    PubMed  CAS  Google Scholar 

  • Thompson, J. N., 1978, Substrates for the assay of ∝-L-iduronidase, Clin. Chim. Acta 89:435.

    PubMed  CAS  Google Scholar 

  • Thompson, J. N., Stoolmiller, A. C., Matalon, R., and Dorfman, A., 1973, N-acetyl-β-hexosaminidase: Role in the degradation of glycosaminoglycans, Science 181:866.

    PubMed  CAS  Google Scholar 

  • Tominaga, F., Oka, K., and Yoshida, H., 1965, The isolation and identification of O-xylosyl-serine and S-methylcysteine sulfoxide from human urine, J. Biochem. 57:717.

    PubMed  CAS  Google Scholar 

  • Tomino, S., Paigen, K., Tulsiani, D. R. P., and Touster, O., 1975, Purification and chemical properties of mouse liver lysosomal (L form) β-glucuronidase, J. Biol. Chem. 250:8503.

    PubMed  CAS  Google Scholar 

  • Toole, B. P., and Lowther, D. A., 1965, The isolation of a dermatan sulfphate-protein complex from bovine heart valves, Biochim. Biophys. Acta 101:364.

    PubMed  CAS  Google Scholar 

  • Toole, B. P., and Lowther, D. A., 1968, Dermatan sulfate-protein: Isolation from and interaction with collagen, Arch. Biochem. Biophys. 128:567.

    PubMed  CAS  Google Scholar 

  • Truppe, W., Basner, R., von Figura, K., and Kresse, H., 1977, Uptake of hyaluronate by cultured cells, Biochem. Biophys. Res. Commun. 78:713.

    PubMed  CAS  Google Scholar 

  • Tsay, G. C., and Dawson, G., 1973, Structure of the “keratosulfate-like” material in liver from a patient with GM1rgangliosidosis (β-d-galactosidase deficiency), Biochem. Biophys. Res. Commun. 52:759.

    PubMed  CAS  Google Scholar 

  • Tulsiani, D. R. P., Keller, R. K., and Touster, O., 1975, The preparation and chemical composition of the multiple forms of β-glucuronidase from the female rate preputial gland, J. Biol. Chem. 250:4770.

    PubMed  CAS  Google Scholar 

  • Turco, S. J., and Heath, E. C., 1977, Glucuronosyl-N-acetylglucosaminyl pyrophosphor-yldolichol: Formation in SV40-transformed human lung fibroblasts and biosynthesis in rat lung microsomal preparations, J. Biol. Chem. 252:2918.

    PubMed  CAS  Google Scholar 

  • von Figura, K., 1977a, Human ∝-N-acetylglucosaminidase. 1. Purification and properties, Eur. J. Biochem. 80:525.

    Google Scholar 

  • von Figura, K., 1977b, Human ∝-N-acetylglucosaminidase. 2. Activity towards natural substrates and multiple recognition forms, Eur. J. Biochem. 80:535.

    Google Scholar 

  • von Figura, K., and Kresse, H., 1972, The Sanfilippo B corrective factor: A N-acetyl-∝-d-glucosaminidase, Biochem. Biophys. Res. Commun. 48:262.

    Google Scholar 

  • von Figura, K., and Kresse, H., 1976, Sanfilippo disease type B: Presence of material cross reacting with antibodies against ∝-N-acetylglucosaminidase, Eur. J. Biochem. 61:581.

    Google Scholar 

  • Wakabayashi, M., 1970, β-Glucuronidases in metabolic hydrolysis, in: Metabolic Conjugation and Metabolic Hydrolysis, Vol. 2 (W. H. Fishman, ed.), p. 520, Academic Press, New York.

    Google Scholar 

  • Wasserman, S. I., and Austen, K. F., 1977, Identification and characterization of arylsul-fatase A and B of the rat basophil leukemia tumor, J. Biol. Chem. 252:7074.

    PubMed  CAS  Google Scholar 

  • Wasteson, Å., Uthne, K., and Westermark, B., 1973, A novel assay for the biosynthesis of sulphated polysaccharide and its application to studies on the effects of somatomedin on cultured cells, Biochem. J. 136:1069.

    PubMed  CAS  Google Scholar 

  • Wasteson, Å., Amado, R., Ingmar, B., and Heldin, C.-H., 1975, Degradation of chondroitin sulphate by lysosomal enzymes from embryonic chick cartilage, in: Protides of the Biological Fluids, 22nd Colloquium (H. Peeters, ed.), p. 431, Pergamon, Oxford.

    Google Scholar 

  • Wasteson, Å., Höök, M., and Westermark, B., 1976, Demonstration of a platelet enzyme, degrading heparan sulphate, FEBS Lett. 64:218.

    PubMed  CAS  Google Scholar 

  • Wasteson, Å., Glimelius, B., Busch, C., Westermark, B., Heldin, C.-H., and Norling, B., 1977, Effect of a platelet endoglycosidase on cell surface associated heparan sulphate of human cultured endothelial and glial cells, Thromb. Res. 11:309.

    PubMed  CAS  Google Scholar 

  • Weissmann, B., and Meyer, K., 1952, Structure of hyaluronic acid: The glucuronidic linkage, J. Am. Chem. Soc. 74:4729.

    CAS  Google Scholar 

  • Weissmann, B., and Meyer, K., 1954, The structure of hyalobiuronic acid and of hyaluronic acid from umbilical cord, J. Am. Chem. Soc. 76:1753.

    CAS  Google Scholar 

  • Weissmann, B., and Santiago, R., 1972, ∝-L-Iduronidase in lysosomal extracts, Biochem. Biophys. Res. Commun. 46:1430.

    PubMed  CAS  Google Scholar 

  • Weissmann, B., Rapport, M. M., Linker, A., and Meyer, K., 1953, Isolation of the aldobionic acid of umbilical cord hyaluronic acid, J. Biol. Chem. 205:205.

    PubMed  CAS  Google Scholar 

  • Weissmann, B., Meyer, K., Sampson, P., and Linker, A., 1954, Isolation of oligosacchar-ides enzymatically produced from hyaluronic acid, J. Biol. Chem. 208:417.

    PubMed  Google Scholar 

  • Weissmann, B., Rowin, G., Marshall, J., and Friederici, D., 1967, Mammalian ∝-acetyl-glucosaminidase. Enzymic properties, tissue distribution and intracellular localization, Biochemistry 6:207.

    PubMed  CAS  Google Scholar 

  • Weissmann, B., Cashman, D. C., and Santiago, R., 1975, Concerted action of β-acetylglucosaminidase on hyaluronodextrins, Connect. Tissue Res. 3:7.

    CAS  Google Scholar 

  • Wolfe, L. S., Senior, R. G., and Kin, N. M. K. N. Y., 1974, The structures of oligosac-charides accumulating in the liver of GM1-gangliosidosis, type I, J. Biol. Chem. 249:1828.

    PubMed  CAS  Google Scholar 

  • Wortman, B., 1961, Enzymic sulfation of corneal mucopolysaccharides by beef cornea epithelial extract, J. Biol. Chem. 236:974.

    PubMed  CAS  Google Scholar 

  • Zechmeister, L., Toth, G., and Vajda, E., 1939, Chromatographie der in der Chitinreihe wirksamen Enzyme der Weinbergschneck, (Helix pomatia), Enzymologia 7:170.

    CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 1980 Plenum Press, New York

About this chapter

Cite this chapter

Rodén, L. (1980). Structure and Metabolism of Connective Tissue Proteoglycans. In: Lennarz, W.J. (eds) The Biochemistry of Glycoproteins and Proteoglycans. Springer, Boston, MA. https://doi.org/10.1007/978-1-4684-1006-8_7

Download citation

  • DOI: https://doi.org/10.1007/978-1-4684-1006-8_7

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4684-1008-2

  • Online ISBN: 978-1-4684-1006-8

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics