LEVEL OF HEMOGLOBIN F AND 
GGAMMA 

GENE EXPRESSION IN SICKLE CELL DISEASE AND THEIR ASSOCIATION WITH HAPLOTYPE AND XMNI POLYMORPHIC SITE IN SOUTH OF IRAN

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RAHIMI Z.; VEYSI RAIGANI A.; MERAT A.; HAGHSHENAS M.

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Journal Paper

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Journal: Iranian Journal of Medical Sciences Year:2007 | Volume:32 | Issue:4 Page(s): 234-239

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Title

LEVEL OF HEMOGLOBIN F AND GGAMMA GENE EXPRESSION IN SICKLE CELL DISEASE AND THEIR ASSOCIATION WITH HAPLOTYPE AND XMNI POLYMORPHIC SITE IN SOUTH OF IRAN

Author(s)

RAHIMI Z. | VEYSI RAIGANI A. | MERAT A. | HAGHSHENAS M. | Issue Writer Certificate

Keywords

HEMOGLOBIN F

GAMMA CHAIN

HAPLOTYPE

SICKLE CELL DISEASE

THALASSEMIA

HEMOGLOBINOPATHY

Abstract

Background: Molecular genetic factors regulating HEMOGLOBIN F (Hb F) expression are important modifiers of the severity of sickle cell anemia (SS).Methods: The prevalence of XmnI polymorphic site, the Gg:Ag ratio and the Hb F level were determined using PCR-RFLP procedure, HPLC and alkaline denaturation method, respectively, in various HAPLOTYPEs of 52 patients with SS, 18 patients with sickle/b-thalassemia (S/Thal), 17 with sickle cell trait (AS) and 53 normal subjects from Fars and Khuzestan provinces who attended the Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran during 2002–03.Results: The prevalence of XmnI (+/+) site in patients with SS was 53.8% which was higher than that for S/Thal (23.5%), AS (22.2%) and normal individuals (7.5%). There was a correlation between the presence of XmnI site and high Gg:Ag ratio in SS and S/Thal patients with Arab-Indian homozygous or heterozygous HAPLOTYPEs (contingency coefficient=0.43, P=0.002). In the present study, the Hb F level was significantly higher in SS patients with one or two Arab-Indian HAPLOTYPEs as compared to Bantu, Benin and Cameroon HAPLOTYPEs. However, the Hb F level was significantly higher in patients with S/Thal having two XmnI sites carrying Arab-Indian and Senegal HAPLOTYPEs as compared to Bantu, Benin and Cameroon HAPLOTYPEs. The increasing effect of presence XmnI site on Hb F level appears only when hemolytic stress is present as in SS and S/Thal patients (contingency coefficient=0.35, P=0.01).Conclusion: The presence of XmnI polymorphic site in HAPLOTYPE backgrounds of Arab-Indian and Senegal in sickle cell anemia is correlated with high level of Hb F and Gg:Ag ratio.

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APA: Copy

RAHIMI, Z., VEYSI RAIGANI, A., MERAT, A., & HAGHSHENAS, M.. (2007). LEVEL OF HEMOGLOBIN F AND ^GGAMMA GENE EXPRESSION IN SICKLE CELL DISEASE AND THEIR ASSOCIATION WITH HAPLOTYPE AND XMNI POLYMORPHIC SITE IN SOUTH OF IRAN. IRANIAN JOURNAL OF MEDICAL SCIENCES (IJMS), 32(4), 234-239. SID. https://sid.ir/paper/276175/en

Vancouver: Copy

RAHIMI Z., VEYSI RAIGANI A., MERAT A., HAGHSHENAS M.. LEVEL OF HEMOGLOBIN F AND ^GGAMMA GENE EXPRESSION IN SICKLE CELL DISEASE AND THEIR ASSOCIATION WITH HAPLOTYPE AND XMNI POLYMORPHIC SITE IN SOUTH OF IRAN. IRANIAN JOURNAL OF MEDICAL SCIENCES (IJMS)[Internet]. 2007;32(4):234-239. Available from: https://sid.ir/paper/276175/en

IEEE: Copy

Z. RAHIMI, A. VEYSI RAIGANI, A. MERAT, and M. HAGHSHENAS, “LEVEL OF HEMOGLOBIN F AND ^GGAMMA GENE EXPRESSION IN SICKLE CELL DISEASE AND THEIR ASSOCIATION WITH HAPLOTYPE AND XMNI POLYMORPHIC SITE IN SOUTH OF IRAN,” IRANIAN JOURNAL OF MEDICAL SCIENCES (IJMS), vol. 32, no. 4, pp. 234–239, 2007, [Online]. Available: https://sid.ir/paper/276175/en

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