Impaired synthesis of heme oxygenase-1 in Fanconi anemia cells can be rescued by transfection of Fanconi wild-type cDNA
| Autor: | Manfred Schweiger, Monica Hirsch-Kauffmann, Maria Kontou |
|---|---|
| Rok vydání: | 2008 |
| Předmět: |
congenital
hereditary and neonatal diseases and abnormalities DNA Complementary Fanconi anemia complementation group C DNA repair Clinical Biochemistry Biology Transfection Biochemistry Cell Line chemistry.chemical_compound Fanconi anemia Cell Line Tumor hemic and lymphatic diseases medicine Humans Molecular Biology Heme Fanconi Anemia Complementation Group A Protein Intracellular Signaling Peptides and Proteins Wild type Membrane Proteins nutritional and metabolic diseases medicine.disease Molecular biology Heme oxygenase Fanconi Anemia chemistry Cancer research Chromosome instability syndrome Heme Oxygenase-1 |
| Zdroj: | bchm. 389:1327-1332 |
| ISSN: | 1437-4315 1431-6730 |
| DOI: | 10.1515/bc.2008.151 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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