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Psoralen-DNA cross-linking photoadducts in dyskeratosis congenita: delay in excision and promotion of sister chromatid exchange.

Abstract
Dyskeratosis congenita is a rare X-linked recessive disease, characterized by mucosal leukokeratosis, nail dystrophy, telangiectasia, reticulated hyperpigmentation, pancytopenia, and a heightened susceptibility to infection and malignancy. We exposed cultured fibroblasts and peripheral leukocytes from normal persons and from 2 unrelated young adult men with dyskeratosis congenita to 4,5',8-trimethylpsoralen and ultraviolet light. We than compared certain of their responses. Labeled DNA from fibroblasts exposed to 4,5',8-trimethylpsoralen and ultraviolet light showed fast-sedimenting DNA, a pattern we interpreted as evidence that cross-linking, psoralen-DNA photoadducts had been formed by the treatment. Fast-sedimenting DNA persisted for 24 hr in dyskeratosis congenita cells but disappeared from normal cells during a 24-hr repair period. Cultured peripheral blood leukocytes from persons with this syndrome similarly exposed to 4,5',8-trimethylpsoralen and ultraviolet light developed more sister chromatid exchanges than did cells from normal persons. These data suggest that a heightened susceptibility in DNA cross-links may be of fundamental importance in the etiology of dyskeratosis congenita.
AuthorsD M Carter, M Pan, A Gaynor, J S McGuire, L Sibrack
JournalThe Journal of investigative dermatology (J Invest Dermatol) Vol. 73 Issue 1 Pg. 97-101 (Jul 1979) ISSN: 0022-202X [Print] United States
PMID448184 (Publication Type: Comparative Study, Journal Article, Research Support, U.S. Gov't, P.H.S.)
Chemical References
  • DNA
  • Trioxsalen
Topics
  • Adolescent
  • Adult
  • Cells, Cultured
  • Chromatids (drug effects, radiation effects)
  • DNA
  • Dose-Response Relationship, Drug
  • Dose-Response Relationship, Radiation
  • Female
  • Fibroblasts (drug effects, radiation effects)
  • Humans
  • Leukocytes (drug effects, radiation effects)
  • Male
  • Photochemotherapy
  • Skin Diseases (blood, congenital, pathology)
  • Trioxsalen (pharmacology)
  • Ultraviolet Rays

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