Document Detail

Beta-globin gene cluster haplotypes in sickle cell patients from southwest Iran.
MedLine Citation:
PMID:  14587041     Owner:  NLM     Status:  MEDLINE    
Sickle cell anemia in Iran is accompanied by a high level of HbF and mild clinical presentation. Here we report haplotypes of the beta gene cluster found in 81 randomly selected sickle cell patients, including 47 sickle cell anemia (SS), 17 sickle cell trait (AS), and 17 sickle/thalassemia (S/thal) from southwest Iran. We found all five common typical haplotypes as well as five atypical haplotypes in our patients. Except for four patients with homozygous Benin haplotype, none of the other African typical haplotypes were found in a homozygous state. Arab-Indian was found to be the most prevalent haplotype in the study population. This haplotype accounted for 51.1% as the homozygous form in SS patients, where 69.1% of chromosomes in these patients had the Arab-Indian haplotype. Bantu A2 was the second most prevalent haplotype among all patients. The mean %HbF in SS patients was 27.83 and in the homozygous Arab-Indian haplotype it was still higher (30.40%), while in AS patients the %HbF was only 1.20. The high %Ggamma chain (71.81) in the Arab-Indian homozygous haplotype was concomitant with the presence of an Xmn I site in both chromosomes. The presence of the Arab-Indian haplotype as the predominant haplotype might be suggestive of a gene flow to/from Saudi Arabia or India. More haplotype investigations of a normal population can clarify the high incidence of Bantu A2 haplotype in our population.
Z Rahimi; M Karimi; M Haghshenass; A Merat
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  American journal of hematology     Volume:  74     ISSN:  0361-8609     ISO Abbreviation:  Am. J. Hematol.     Publication Date:  2003 Nov 
Date Detail:
Created Date:  2003-10-30     Completed Date:  2003-12-12     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  7610369     Medline TA:  Am J Hematol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  156-60     Citation Subset:  IM    
Copyright Information:
Copyright 2003 Wiley-Liss, Inc.
Department of Biochemistry, Medical School, Shiraz University of Medical Sciences, Shiraz, Iran.
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MeSH Terms
Anemia, Sickle Cell / genetics*
Child, Preschool
Globins / genetics*
Haplotypes / genetics*
Hematologic Tests
Hemoglobin, Sickle / genetics
Iran / ethnology
Middle Aged
Multigene Family / genetics
Polymorphism, Restriction Fragment Length
Sickle Cell Trait / genetics
Thalassemia / genetics
Reg. No./Substance:
0/Hemoglobin, Sickle; 9004-22-2/Globins

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