The Response of Cockayne Syndrome Cells to UV-Irradiation

  • Alan R. Lehmann
  • Lynne Mayne
Part of the NATO Advanced Study Institutes Series book series (NSSA, volume 40)


Cockayne syndrome (CS) is a rare autosomal recessive disorder. The principal clinical features are dwarfism with a small head and senile appearance, skeletal abnormalities, loss of adipose tissue, severe mental retardation, retinal degeneration and severe photosensitivity. The onset occurs after several months of apparently normal development. The retinal, neurological and mental symptoms are progressive, as is the loss of adipose tissue1. There are no reports in the literature of elevated frequencies of cancer, as found for example in xeroderma pigmentosum. With one or two exceptions the karyotype is normal.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    F. Guzzetta, in “Handbook of Clinical Neurology”, P. J. Vinken and G. W. Bruyn, Vol.13, pp.431–440, Amsterdam: North Holland Publishing Co. (1972).Google Scholar
  2. 2.
    R. D. Schmickel, E. H. Y. Chu, J. E. Trosko and C. C. Chang, Paediatrics 60: 135–139 (1977).Google Scholar
  3. 3.
    A. D. Andrews, S. F. Barrett, F. W. Yoder and J. H. Robbins, J. Invest. Dermatol. 70: 237–239 (1978).CrossRefGoogle Scholar
  4. 4.
    R. R. Marshall, C. F. Arlett, S. A. Harcourt and B. C. Broughton, Mutation Res., 69: 107–112 (1980).CrossRefGoogle Scholar
  5. 5.
    M. H. Wade and E. H. Y. Chu, Mutation Res. 59: 49–60 (1979).CrossRefGoogle Scholar
  6. 6.
    R. S. Day and C. Ziolkowski, in “DNA Repair Mechanisms” P. C. Hanawalt, E. C. Friedberg and C. F. Fox, pp.535–539, New York, Academic Press (1978).Google Scholar
  7. 7.
    D. I. Hoar and F. Davis, Mutation Res., 62: 401–405 (1979).CrossRefGoogle Scholar
  8. 8.
    W.-S. Cheng, R. E. Tarone, A. D. Andrews, J. S. Whang-Peng and J. H. Robbins, Cancer Res. 38: 1601–1609 (1978).PubMedGoogle Scholar
  9. 9.
    F. E. Ahmed and R. B. Setlow, Biochim. Biophys. Acta 521: 805–817 (1978).CrossRefGoogle Scholar
  10. 10.
    A. R. Lehmann, S. Kirk-Bell and L. Mayne, Cancer Res., 39: 42374241 (1979).Google Scholar
  11. 11.
    J. M. Rudd and E. C. Friedberg, Mutation Res., 42: 433–442 (1977).CrossRefGoogle Scholar
  12. 12.
    G. J. Kantor, C. Warner and D. R. Hull, Photochem. Photobiol. 25: 483–489 (1977).CrossRefGoogle Scholar

Copyright information

© Plenum Press, New York 1981

Authors and Affiliations

  • Alan R. Lehmann
    • 1
  • Lynne Mayne
    • 1
  1. 1.MRC Cell Mutation UnitUniversity of SussexFalmerEngland

Personalised recommendations