Objective: To evaluate an assocn. of Bloom syndrome (BS) phenotype with an in vivo pro-oxidant state. Methods: The following endpoints were measured in 4 BS patients, their 6 parents, and 78 controls: a. leukocyte and urinary 8-hydroxy-2'-deoxyguanosine (8-OHdG); b. blood glutathione (GSSG and GSH), c. plasma levels of some plasma antioxidants (uric acid, UA, ascorbic acid, AA, a- and g-tocopherol), and of glyoxal (Glx) and methylglyoxal (MGlx). Results: Leukocyte 8-OHdG levels were significantly increased in the 4 BS patients vs. 40 controls (p = 0.04), while the urinary 8-OHdG levels were non-significantly increased in BS patients. Glutathione disulfide levels and GSSG/GSH ratio were significantly decreased in BS patients vs. 44 controls (p = 0.02). The plasma levels of UA in BS patients were significantly increased vs. 24 controls (p = 0.005). No significant alterations were found in the in the plasma levels of Glx, MGlx, AA, and tocopherol. No changes in the tested parameters were found in the BS heterozygotes. Conclusion: This report shows a significant increase in oxidative DNA damage in leukocytes and in plasma UA levels from 4 BS patients. Should these data be confirmed in more extensive BS patient groups, an involvement of oxidative stress in the clin. BS phenotype might be suggested.

Oxidative stress biomarkers in four Bloom syndrome (BS) patients and in their parents suggest in vivo redox abnormalities in BS phenotype

D'ISCHIA, MARCO;
2007

Abstract

Objective: To evaluate an assocn. of Bloom syndrome (BS) phenotype with an in vivo pro-oxidant state. Methods: The following endpoints were measured in 4 BS patients, their 6 parents, and 78 controls: a. leukocyte and urinary 8-hydroxy-2'-deoxyguanosine (8-OHdG); b. blood glutathione (GSSG and GSH), c. plasma levels of some plasma antioxidants (uric acid, UA, ascorbic acid, AA, a- and g-tocopherol), and of glyoxal (Glx) and methylglyoxal (MGlx). Results: Leukocyte 8-OHdG levels were significantly increased in the 4 BS patients vs. 40 controls (p = 0.04), while the urinary 8-OHdG levels were non-significantly increased in BS patients. Glutathione disulfide levels and GSSG/GSH ratio were significantly decreased in BS patients vs. 44 controls (p = 0.02). The plasma levels of UA in BS patients were significantly increased vs. 24 controls (p = 0.005). No significant alterations were found in the in the plasma levels of Glx, MGlx, AA, and tocopherol. No changes in the tested parameters were found in the BS heterozygotes. Conclusion: This report shows a significant increase in oxidative DNA damage in leukocytes and in plasma UA levels from 4 BS patients. Should these data be confirmed in more extensive BS patient groups, an involvement of oxidative stress in the clin. BS phenotype might be suggested.
2007
Bloom syndrome; oxidative stress; glyoxal; methylglyoxal
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11384/83980
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