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

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• 作者：Adriana Zatterale ; Frank J. Kelly ; Paolo Degan ; Marco d'Ischia ; Federico V. Pallardó ; Rita Calzone ; Christina Dunster ; Ana Lloret ; Paola Manini ; Ozgur Coğ ; ulu ; Kaan Kavaklı ; ; Giovanni
• 关键词：Bloom syndrome ; Oxidative stress ; 8-hydroxy-2′ ; -deoxyguanosine ; Glutathione ; Uric acid ; Ascorbic acid ; Glyoxal ; Methylglyoxal
• 刊名：Clinical Biochemistry
• 出版年：2007
• 出版时间：October 2007
• 年：2007
• 卷：40
• 期：15
• 页码：1100-1103
• 全文大小：136 K

文摘

To evaluate an association of Bloom syndrome (BS) phenotype with an in vivo prooxidant 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, α- and γ-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 clinical BS phenotype might be suggested.