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Increased rate of superoxide ion generation in Fanconi anemia erythrocytes
Authors:M Scarpa  A Rigo  F Momo  G Isacchi  G Novelli  B Dallapiccola
Institution:1. Institute of Biological Chemistry, University of Padova, Padova, Italy;4. Chair of Hematology, 1° University of Rome, Rome, Italy;9. Chair of Genetics, University of Urbino, Urbino, Italy;99. Chair of Genetics, 2° University of Rome, Rome, Italy;1. Department of Haematology, Sultan Qaboos University Hospital, Muscat, PC123, Oman;2. College of Medicine and Health Sciences, Sultan Qaboos University, Muscat, PC123, Oman;3. Department of Pathology, Sultan Qaboos University Hospital, Muscat, PC123, Oman;4. Child Health Department, Sultan Qaboos University Hospital, Muscat, Oman;5. Department of Pediatrics, Faculty of Medicine, Alexandria University, Alexandria, Egypt
Abstract:The rate of generation of superoxide ion, the concentration of Cu, Zn superoxide dismutase and the hematological parameters were measured in red blood cells obtained from Fanconi anemia patients and from healthy individuals. No significative difference in the superoxide dismutase concentration was found, while the rate of generation of the superoxide ion doubled in Fanconi anemia patients. The steady-state concentration of the superoxide ion was calculated from these data and was found to be 2.3 times higher in Fanconi anemia erythrocytes than in controls. The possible consequences with respect to the alterations in FA are discussed.
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