A Comparison of Adult and Childhood Progerias: Werner Syndrome and Hutchinson-Gilford Progeria Syndrome

  • W. Ted Brown
  • Fred J. Kieras
  • George E. HouckJr.
  • Regina Dutkowski
  • Edmund C. Jenkins
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 190)


The Werner syndrome, also known as progeria of the adult, and the childhood Hutchinson-Gilford Progeria Syndrome (hereafter Progeria), both serve as genetic disease models of human aging (Brown, 1979). A comparison of their similarities and differences may be useful in order to gain insight into the nature of the genetic mutations underlying these conditions. Their modes of inheritance indicate the involvement of a single gene. This implies that some specific genes may lead to a phenotype of greatly accelerated senescence and that such genes may have direct effects on the rate of aging. Determining the basic mechanisms involved in producing their phenotypes may point the way to an understanding of important pathogenetic aspects underlying the aging process.


Hyaluronic Acid Uronic Acid Werner Syndrome Sister Chromatid Exchange Frequency Crouzon Syndrome 
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  1. Brown, W.T., 1979, Human mutations affecting aging - a review, Mechanisms of Aging and Development, 9: 325.CrossRefGoogle Scholar
  2. Brown, W.T., Ford, J., and Gersey, E.L., 1980a, Variation of DNA repair in Progeria Cells unrelated to growth conditions, Biochemical and Biophysical Research Communications, 97:347.Google Scholar
  3. Brown, W.T., Darlington, G.J., 1980b, Thermolabile in progeria and Werner Syndrome: Evidence contrary to the protein error hypothesis, Am. J. Hum. Genet., 32:614.PubMedGoogle Scholar
  4. Brown, W.T., Darlington, G.J., Fotino, M., and Arnold, A., 1980c, Detection of HLA antigens on progeria fibroblasts, Clinical Genetics, 17: 213.PubMedCrossRefGoogle Scholar
  5. Damle, S.P., Kieras, F.J., Tzeng, W.-K., and Gregory, J.D., 1979, Isolation and characterization of proteochondroitin sulfate from pig skin, J. Biol. Chem., 254: 1614.Google Scholar
  6. Darlington, G.J., Dutkowski, R., Brown, W.T., 1981, Sister chromatid exchange frequencies in progeria and Werner syndrome patients, Am. J. Hum. Genet., 33: 762.Google Scholar
  7. DeBusk, F.L., 1972, The Hutchinson-Gilford progeria syndrome, J. Pediat., 697.Google Scholar
  8. Elson, L.A., and Morgan, W.T.J., 1933, A colorimetric method for the determination of glucosamine and chondrosamine, Biochem. J., 27: 1824.Google Scholar
  9. Epstein, C.J., Martin, G.M., Schultz, A.L., Motulsky, A., 1966, Werner’s syndrome: a review of its symptomatology, natural history, pathological features, genetics and relationship to the natural aging process, Medicine, 45: 177.PubMedGoogle Scholar
  10. Erecinski, K., Bittel-Dobryzynska, N., Mostowiec, S., 1961, Zespol progerii u dwoch braci, Pol. Tyrg, Lek., 16: 806.Google Scholar
  11. Franklin, P.P., 1976, Progeria in siblings, Clin. Radiol., 27: 327.Google Scholar
  12. Gabr, M., Hashem, M., Fahmi, A., Safouh, M., 1960, Progeria, a pathologic study, J. Pediat., 57: 70.PubMedCrossRefGoogle Scholar
  13. Goto, M., Tanimoto, K., Horuichi, Y., Sasuzuji, T., 1981, Family analysis of Werner’s syndrome: A survey of 42 Japanese families with a review of the literature, Clin. Genet., 19: 8.Google Scholar
  14. Hall, C.W., Cantz, M., Neufeld, E.F., 1973, A beta-glucuronidase deficiency mucopolysaccharidosis: Studies in cultured fibroblasts, Arch. Biochem. Biophys., 155: 32.Google Scholar
  15. Hoehn, H., Bryant, E.M., Au, K., Norwood, T.H., Boman, H., Martin, G.M., 1975, Variegated translocation mosaicism in human skin fibroblast cultures, Cyto. Cell Genet., 15: 282.Google Scholar
  16. Jones, K.L., Smith, P.W., Harvey, M.A.S., Hall, B.D., Quan, L., 1975, Older paternal age and fresh gene mutation: Data on additional disorders, J. Pediat., 86: 84.Google Scholar
  17. Littlefield, L.G., Mailhes, J.B., 1975, Observations of de novo clones of cytogenetically aberrant cells in primary fibroblast cell strains from phenotypically normal women, Am. J. Hum. Genet., 27: 190.Google Scholar
  18. Martin, G.M., 1977, Genetic syndromes in man with potential relevance to the pathobiology of aging, Birth Defects Orig. Article Series, Genetics of Aging, 14: 5.Google Scholar
  19. McKusick, V.A., 1983, “Mendelian inheritance in man, catalogs of autosomal dominant, autosomal recessive and X-linked phenotypes,” 7th Edn., Johns Hopkins Univ. Press, Baltimore.Google Scholar
  20. Norwood, T.H., Hoehn, H., Salk, D., Martin, G.M., 1979, Cellular aging in Werner’s syndrome: a unique phenotype? J. Invest. Derm., 73: 92.Google Scholar
  21. Nordenson, I., 1977, Chromosome breaks in Werner’s syndrome and their prevention in vitro by radical-scavenging enzymes, Hereditas, 87: 151.PubMedCrossRefGoogle Scholar
  22. Okada, S., Veath, M.L., Lerry, J., O’Brien, J.S., 1971, Ganglioside GM2 storage diseases: Hexosaminidase deficiencies in cultured fibroblasts, Amer. J. Hum. Genet., 23: 55.Google Scholar
  23. Orkin, R.W., Underhill, C.B., Toole, B.P., 1982, Hyaluronate degradation in 3T3 and Simian virus-transformed 3T3 cells, J. Biol. Chem., 257: 5821.Google Scholar
  24. Rautenstruauch, T. and Snigula, F., 1977, Progeria: A cell culture study and clinical report of familial incidence, Europ. J. Pediat., 124: 101.Google Scholar
  25. Raya, G., 1967, Su un nucleo familiare di progeria, Minerva Med., 58: 1502.Google Scholar
  26. Salk, D., Au, K., Hoehn, H., Martin, G.M., 1981, Cytogenetics of Werner’s syndrome cultured skin fibroblasts: variegated translocation mosaicism, Cytogenet. Cell Genet., 30: 92.Google Scholar
  27. Salk, D., 1982, Werner Syndrome: A review of recent research with an analysis of connective tissue metabolism, growth control of cultured cells, and chromosomal aberrations, Hum. Genet., 62: 1.Google Scholar
  28. Scappaticci, S., Cerimele, D., Fraccaro, M., 1982, Clonal structural chromosomal rearrangements in primary fibroblast cultures and in lymphocytes of patients with Werner’s syndrome, Hum. Genet., 62: 16.Google Scholar
  29. Schiller, S., Slover, G.A., Dorfman, A., 1961, A method for the separation of acid mucopolysaccharides: Its application to the isolation of heparin from the skin of rats, J. Biol. Chem., 236: 983.Google Scholar
  30. Schonberg, S., Henderson, E., Niermeijev., M.F., Bootsma, D., Gérman, J., 1982, Werner’s syndrome: preferential proliferation of clones with chromosome translocations, MS submitted for publication. Schonberg, S.A., Henderson, E., Niermeijer, M.F., German, J.,1981, Werner’s syndrome: preferential proliferation of clones with translocations, Am., J. Hum. Genet., 33: 120A.Google Scholar
  31. Tokunaga, M., Wakamatsu, E., Sato, K., Satake, S., Aoyama, K., Saito, K., Sugawara, M., Yosizawa, Z., 1978, Hyaluronuria in a case of progeria ( Hutchinson-Gilford Syndrome)., J. Amer. Geriatrics Soc., 26: 296.Google Scholar
  32. Varadi, D.P., Cifonelli, J.A., Dorfman, A., 1967, The acid mucopolysaccharides in normal urine, Biochim, Biophys. Acta, 141: 103.Google Scholar

Copyright information

© Plenum Press, New York 1985

Authors and Affiliations

  • W. Ted Brown
    • 1
  • Fred J. Kieras
    • 1
  • George E. HouckJr.
    • 1
  • Regina Dutkowski
    • 1
  • Edmund C. Jenkins
    • 1
  1. 1.The New York State Institute for Basic Research in Developmental Disabilities, Staten IslandNew York and Cornell University Medical CollegeNew YorkUSA

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