Cross-sensitivity of certain xeroderma pigmentosum and cockayne syndrome fibroblast strains to both ionizing radiation and ultraviolet light

1981 ◽  
Vol 181 (4) ◽  
pp. 562-563 ◽  
Author(s):  
Gerald L. Chan ◽  
John B. Little
Keyword(s):  
Ionizing Radiation ◽  
Ultraviolet Light ◽  
Xeroderma Pigmentosum ◽  
Cockayne Syndrome ◽  
Cross Sensitivity

scholarly journals An Xpd mouse model for the combined xeroderma pigmentosum/Cockayne syndrome exhibiting both cancer predisposition and segmental progeria

Cancer Cell ◽  
2006 ◽  
Vol 10 (2) ◽  
pp. 121-132 ◽  
Author(s):  
Jaan-Olle Andressoo ◽  
James R. Mitchell ◽  
Jan de Wit ◽  
Deborah Hoogstraten ◽  
Marcel Volker ◽  
...  
Keyword(s):  
Mouse Model ◽  
Xeroderma Pigmentosum ◽  
Cockayne Syndrome ◽  
Cancer Predisposition

Cockayne Syndrome: A Cellular Sensitivity to Ultraviolet Light

PEDIATRICS ◽  
1977 ◽  
Vol 60 (2) ◽  
pp. 135-139
Author(s):  
R. D. Schmickel ◽  
E. H. Y. Chu ◽  
J. E. Trosko ◽  
C. C. Chang
Keyword(s):  
Ultraviolet Light ◽  
Uv Light ◽  
Cockayne Syndrome ◽  
Fibroblast Cultures ◽  
Cellular Sensitivity ◽  
X Irradiation ◽  
Increased Sensitivity ◽  
Cellular Dna ◽  
Thymidine Dimer ◽  
Enzymatic Defect

Two unrelated children, a boy 2½ 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.

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