Deficiency of DNA ligase activity in Fanconi's anemia |
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| Authors: | Monica Hirsch-Kauffmann Manfred Schweiger Erwin F Wagner Karl Sperling |
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| Institution: | (1) Max-Planck-Institut für Molekulare Genetik, D-1000 Berlin-Dahlem, Germany;(2) Max-Planck-Institut für Zellbiologie, D-2940 Wilhelmshaven, Germany;(3) Institut für Biochemie (nat. Fak.), Universität Innsbruck, Austria;(4) Institut für Humangenetik, Freie Universität Berlin-Charlottenburg, D-1000 Berlin, Germany |
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| Abstract: | Summary A significant decrease in DNA ligase activity was observed in lymphocytes and fibroblasts of a patient with Fanconi's anemia (FA). This decrease is related to the observed DNA repair deficiency indicated by the delayed closing of repair DNA strands following UV irradiation. Other steps of DNA repair were analyzed in the FA fibroblasts, including endonucleolytic incision of DNA, repair DNA synthesis, and exonucleolytic removal of the photoproducts. No differences were found against control cells. The action of DNA ligase is delayed during replication in the FA cells, as seen by an accumulation of replicative intermediates. |
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