PEDIATRICS Vol. 60 No. 2 August 1977, pp. 135-139
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Cockayne Syndrome: A Cellular Sensitivity to Ultraviolet Light

R. D. Schmickel M.D.1, E. H. Y. Chu Ph.D.1, J. E. Trosko Ph.D.1, and C. C. Chang Ph.D.1

1 Departments of Pediatrics and Communicable Diseases and Human Genetics, The University of Michigan, Ann Arbor. and the Department of Human Development, Michigan State University, East Lansing

Two unrelated children, a boy 2frac12 years old and a girl 4 years old, were affected with the cachectic dwarfism of Cockayne syndrome. Fibroblast cultures derived from these patients exhibited increased sensitivity to ultraviolet (UV) light, but not to x-irradiation, as measured by colony-forming ability. In both Cockayne fibroblast cultures, the rate of removal of thymidine dimer from the irradiated cellular DNA was normal. This demonstration of a cellular defect in Cockayne cells suggests that there may be an enzymatic defect in the repair of UV light-induced damage.

Submitted on April 16, 1976
Accepted on January 21, 1977




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