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Acidic Glycosaminoglycans in Werner’s Syndrome: Studies on Levels in Tissue, Organ, Cell, and Fluid

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Werner’s Syndrome and Human Aging

Part of the book series: Advances in Experimental Medicine and Biology ((AEMB,volume 190))

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Abstract

The clinical manifestations of Werner’s syndrome resemble those of the normal aging process (Werner, 1904; Epstein et al., 1966; Murata and Nagashima, 1982). Scleroderma-like features and cataracts are the main symptoms of Werner’s syndrome in the original description (Werner, 1904). It is a genetic disorder disease affecting the mesodermal connective tissues of the whole body (Fleischmajer and Nedwich, 1973) of which acidic glycosaminoglycans (AGAG) are one component. Some of the degraded components of these AGAG are excreted in urine as catabolic products. In normal subjects, urinary AGAG comprise small amounts of chondroitin 4-sulfate (C-4S), chondroitin 6-sulfate (C-6S), and heparan sulfate (HS) and even smaller amounts of other chondroitin sulfate (CS) isomers (Varadi, 1967; Murata et al., 1971; Wessler, 1971; Taniguchi, 1972). Hyaluronic acid (HA) is absent or in trace amounts (Varadi et al., 1967).

Supported in part by Grants-in-aid from the Ministry of Education, Science and Culture of Japan, and grants from the Life Science Section of the Institute of Physical and Chemical Research and from the Adult Disease Clinic Memorial Foundation, Tokyo.

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References

  • Arai, H., Kang, K., Saito, H., Nagai, H., Motomiya, M., and Konno, K., 1979, Significance of the quantification and demonstration of hyaluronic acid in tissue specimens for the diagnosis of pleural mesothelioma, Am. Rev. Resp. Dis., 120: 529.

    PubMed  CAS  Google Scholar 

  • Bitter, J., and Muir, H., 1962, A modified uronic acid carbazole reaction, Analyt. Biochem., 4: 330.

    Article  PubMed  CAS  Google Scholar 

  • Epstein, C.J., Martin, G.M., Schultz, A.L., and Motulsky, A.G., 1966, Werner’s syndrome. A review of its symptomatology, natural history, pathologic features, genetics and relationship to the natural aging process, Medicine, 45: 177

    PubMed  CAS  Google Scholar 

  • Fleischmajer, R., and Nedwich, A., 1973, Werner’s syndrome, Am. J. Med., 54: 111.

    Article  PubMed  CAS  Google Scholar 

  • Fleischmajer, R., Perlish, J.S., and Bashey, R.I., 1972, Human dermal glycosaminoglycans and aging, Biochim. Biophys. Acta, 279: 265.

    Article  PubMed  CAS  Google Scholar 

  • Fleischmajer, R., Perlish, J.S., and Gaisin, A., 1973, Comparative study of dermal glycosaminoglycans, J. Invest. Derm., 61: 1.

    Article  PubMed  CAS  Google Scholar 

  • Goto, M., and Murata, K., 1978, Urinary excretion of macrolomecular acidic glycosaminoglycans in Werner’s syndrome, Clin. Chin. Acta, 85: 101.

    Article  CAS  Google Scholar 

  • Kojima, J., Nakamura, N., Kanatani, M., and Ohmori, K., 1975, The glycosaminoglycans in human hepatic cancer, Cancer Res, 35: 542.

    PubMed  CAS  Google Scholar 

  • Laurent, T.C., 1970, Structure of hyaluronic acid, in: “Chemistry and Molecular Biology of the Intercellular Matrix,” E.A. Balaazs, ed., Academic Press, New York, p. 703.

    Google Scholar 

  • Masuda, H., Shichijo, S., and Takeuchi, M., 1977, Comparative studies on the distribution of the glycopeptide and glycosaminoglycans in the human abdominal wall, Int. J. Biochem., 8: 633.

    Article  CAS  Google Scholar 

  • Murata, K., 1979, Differences in acidic glycosaminoglycans in cortical and medullary tissue in human kidney, Renal Physiol, 2: 346.

    Google Scholar 

  • Murata, K., 1981, Acidic glycosaminoglycans in human heart values, J. Mol. Cell. Cardiol., 13: 281.

    Article  PubMed  CAS  Google Scholar 

  • Murata, K., 1980, Enzymic assay of acidic glycosaminoglycans, in: “Methods in Carbohydrate Chemistry,” R.L. Whistler and J.N. BeMiller, eds., Academic Press, New York, p. 79.

    Google Scholar 

  • Murata, K., 1982, Glycosaminoglycans in kidney normal and pathological aspects, in: “Glycosaminoglycans and proteoglycans in Physiological and Pathological Processes of Body System,” R.S., Varma, R. Varma, eds., Karger, Warren Pa.

    Google Scholar 

  • Murata, K., Akashio, K., and Ochiai, Y., 1984, Changes in acidic glycosaminoglycan components at different stages of human liver cirrhosis, Hepato-Gastroent., in press.

    Google Scholar 

  • Murata, K., and Horiuchi Y., 1977, Molecular weight-dependent distribution of acidic glycosaminoglycans in human plasma, Clin. Chini. Acta, 75: 59.

    Article  CAS  Google Scholar 

  • Murata, K., and Nakazawa, K., 1976, Composition of acidic glycosaminoglycans in human cerebral arteries, Atherosclerosis., 25: 31.

    Article  PubMed  CAS  Google Scholar 

  • Murata, K., Nakazawa, K., and Hamai, A, 1975, Distribution of acidic glycosaminoglycans in the intima, media and adventitia of bovine aorta and their anticoagulant properties, Atherosclerosis, 21: 93.

    Article  PubMed  CAS  Google Scholar 

  • Murata, K., and Nakashima, H., 1982, Werner’s syndrome: Twenty-four cases with a review of the Japanese medical literature, Am. Geriat. Soc., 30: 303.

    CAS  Google Scholar 

  • Murata, K., Ishikawa, T., and Ninomiya, H., Age-dependent changes of chondroitin sulfate isomers in normal human urine, Biochem. Med., 8: 472.

    Google Scholar 

  • Murata, K., Ogura, T., and Okuyama, T., 1974, The acidic glycosaminoglycAns in leukocytes: an application of enzymatic methods, Connect. Tiss. Res., 2: 101.

    Article  CAS  Google Scholar 

  • Murata, K., Okudaira, M., and Adashio, K., 1973, Mast cells in human liver tissue: increased mast cell number in relation to the components of connective tissue in cirrhotic process, Acta Dermatoverner, 73: 157.

    CAS  Google Scholar 

  • Murata, K., and Takeda, M., 1980, Compostional changes of urinary acidic glycosaminoglycans in progressive systemic sclerosis, Clin. Chim. Acta, 108: 49.

    Article  PubMed  CAS  Google Scholar 

  • Murata, K., and Yokoyama, Y., 1982, Acidic glycosaminoglycan, lipid and water content in human coronary arterial branches, Atherosclerosis, 45: 53.

    Article  PubMed  CAS  Google Scholar 

  • Nakazawa, K., and Murata, K., 1975, Acidic glycosaminoglycans in three layers of human aorta: their different constitution and anticoacul and function, Arterial Wall, 2: 203.

    PubMed  CAS  Google Scholar 

  • Praag, D.V., Stone, A.L., Richter, A.J., and Farber, S.J., 1972, Composition of glycosaminoglycans (mucopolysaccharides) in rabbit kidney. II Renal cortex, Biochim. Biophys. Acta, 273: 149.

    Article  PubMed  Google Scholar 

  • Reichel, W., Garcia-Bunnel, R., and Dilallo, J., 1971, Progería and Werner’s syndrome as models for the study of normal human aging, J. Am, Geriatr. Soc., 19: 369.

    CAS  Google Scholar 

  • Saito, H., Yamagata, T., and Suzuki, S., 1968, Enzymatic methods for the determination of small quantities of isomeric chondroitin sulfates, J. Biol. CHem., 243: 1536.

    PubMed  CAS  Google Scholar 

  • Sekino, T., and Murata, K., 1978, Age dependent chostitutional change of acidic glycosaminoglycans in human esophagus, Digestion, 18: 319.

    Article  PubMed  CAS  Google Scholar 

  • Seno, N., Anno, K., Kondo, K., Nagase, S., and Saito, S., 1970, improved method for electrophoretic separation and rapid quantitation of isomeric chondroitin sulfates on celllulose acetate strips, Analyt. Biochem., 37: 197.

    Article  PubMed  CAS  Google Scholar 

  • Shelter, M.R., Shetler, C.L., Chien, S.F., Linares, H., Dobrokowsky, M., and Larson, D.L., 1972, The hypertrophic scar. Hexosamine containing components of burn scars, Proc. Soc. Biol. Med., 139: 544.

    Google Scholar 

  • Takeuchi, J., Sobue, M., Shimamoto, M., Yoshida, M., Sato, E., and Leighton, H., 1977, Cell surface glycosaminoglycans of cell line MDCK derived from canine kidney, Cancer Res., 37: 1507.

    PubMed  CAS  Google Scholar 

  • Taniguchi, N., 1972, Age differences in the pattern of urinary glycosaminoglycan excretion in normal individuals, Clin. Chini. Acta, 37: 225.

    Article  CAS  Google Scholar 

  • Tokunaga, M., Futami, T., Wakamatsu, Endo, M. and yoshizawa, Z., 1975, Werner’s syndrome as hyaluronuria, Clin. Chini. Acta, 62: 89.

    Article  CAS  Google Scholar 

  • Varadi, D.P., Cifonelli, J.A., and dorfman, A., 1967, The acid mucopolysaccharides in normal human urine, Biochim. Biophys. Acta, 141: 103.

    Article  PubMed  CAS  Google Scholar 

  • Werner, C.W.O., 1904, Uber Katarakt in Verbindung mit sklerodermie, Thesis, Schmidt and Klauning, Kiel, Doctoral dissertation.

    Google Scholar 

  • Wessler, E., 1968, Analytical and preparative separation of acid glycosaminoglycans by electrophoresis in barium acetate, Analyt. Biochem., 26: 439.

    Article  PubMed  CAS  Google Scholar 

  • Wessler, E., 1971, The nature of the non-ultrafiltrable glycosaminoglycans of normal human urine, Biochem. J., 112: 373.

    Google Scholar 

  • Yamada, Y., Murata, K., Miyamoto, Y., and Horiuchi, Y., 1979, 2 cases of Werner’s syndrome with hyaluronuria, Metabolism, ( Tokyo ), 16: 1215 (in Japanese)

    Google Scholar 

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Author information

Authors and Affiliations

  1. Department of Medicine and Physical Therapy Faculty of Medicine, University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113, Japan

    Katsumi Muratal

  2. The Third Department of Medicine, Kagoshima University School of Medicine, Kagoshima, Japan

    Ryoichi Hiwatari

  3. Institute of Medical Science, University of Tokyo, Japan

    Toshiharu Matsumura

Authors
  1. Katsumi Muratal
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  2. Ryoichi Hiwatari
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  3. Toshiharu Matsumura
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Editor information

Editors and Affiliations

  1. School of Medicine, University of Washington, Seattle, Washington, USA

    Darrell Salk  & George M. Martin  & 

  2. Kobe University School of Medicine, Kobe, Japan

    Yoshisada Fujiwara

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© 1985 Plenum Press, New York

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Muratal, K., Hiwatari, R., Matsumura, T. (1985). Acidic Glycosaminoglycans in Werner’s Syndrome: Studies on Levels in Tissue, Organ, Cell, and Fluid. In: Salk, D., Fujiwara, Y., Martin, G.M. (eds) Werner’s Syndrome and Human Aging. Advances in Experimental Medicine and Biology, vol 190. Springer, Boston, MA. https://doi.org/10.1007/978-1-4684-7853-2_31

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  • DOI: https://doi.org/10.1007/978-1-4684-7853-2_31

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4684-7855-6

  • Online ISBN: 978-1-4684-7853-2

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