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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

Abstract

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