Annals of Human Biology
Short Report
DNA damage: beta zero versus beta plus thalassemia
Posted online on December 26, 2014. (doi:10.3109/03014460.2014.990921)
Chandan S. Sagar, Rakesh Kumar, Dharmesh C. Sharma, and Purnima Kishor
1School of Studies in Biochemistry, Jiwaji University, Gwalior , India and
2Blood Bank, Jaya Arogya Hospitals, Gajra Raja Medical College, Gwalior , India
Correspondence:
Purnima Kishor , School of Studies in Biochemistry, Jiwaji University, Gwalior , India. Tel: (0) 917512442795, 919425110380. E-mail:
[email protected]
Abstract
Beta thalassemia results in an increase in the alpha to non-alpha chain ratio. The unpaired alpha chains precipitate in the form of Heinz bodies. Regular transfusions leading to iron overload and the release of iron from the unpaired alpha chains in RBCs are the major cause of cellular damage in beta thalassemics. The use of iron chelators to counter the iron overload is accompanied by side-effects. The present work demonstrates a correlation between beta globin gene mutation and oxidative stress in beta thalassemia patients of the Gwalior Chambal region of central India. While Heinz bodies and nucleated RBCs were seen in all the patients, oxidation of 2'7' dichlorofluorescin (DCFH) and DNA damage were seen to be associated with the beta globin gene defect. Single cell gel electrophoresis has revealed that DNA damage is greater in the blood cells of patients homozygous for beta zero mutations when compared with patients with beta plus thalassemia. Maximum DNA degradation was seen in patients presenting with the 619 base pair deletion. Monitoring of the dose of iron chelators, according to the type of mutation in the beta globin gene, may help improve the compliance of beta thalassemics to chelation therapy and prevent side-effects in patients with beta plus mutations.
Keywords
Alpha chain toxicity, DCFH, oxidative stress
Read More: http://informahealthcare.com/doi/abs/10.3109/03014460.2014.990921
