Skip to content
Licensed Unlicensed Requires Authentication Published by De Gruyter August 19, 2008

Impaired synthesis of heme oxygenase-1 in Fanconi anemia cells can be rescued by transfection of Fanconi wild-type cDNA

  • Maria Kontou , Monica Hirsch-Kauffmann and Manfred Schweiger
From the journal Biological Chemistry


Fanconi anemia is a fatal, hereditary chromosome instability syndrome of early childhood with progressive pancytopenia and cancer-proneness. Hypersensitivity to alkylating agents points to DNA repair inefficiency. Excess reactive oxygen intermediates and hypersensitivity to oxygen, all features of Fanconi anemia cells, give evidence for a disturbed oxidative metabolism. Here, we report that expression of the inducible heme oxygenase-1, an essential antioxidative defense protein, is impaired in Fanconi anemia cells and can be reinstated with the transfection of Fanconi A wild-type cDNA. A causative interaction of Fanconi anemia proteins with transcription of selected proteins is indicated. The results enlighten the oxygen sensitivity in Fanconi anemia.

Corresponding author

Received: 2008-4-22
Accepted: 2008-7-4
Published Online: 2008-08-19
Published in Print: 2008-10-01

©2008 by Walter de Gruyter Berlin New York

Downloaded on 28.9.2023 from
Scroll to top button