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Combined ?-thalassemia and Hemoglobin J-Iran (?77 His ? Asp). A Family Study in southern Iran

AUTHORS

SJ Dehghani 1 , A Amiri Dashtarzhen 1 , Sh Nasirabadi 1 , J Dehbozorgian 1 , AR Afrasiabi 1 , N Morshedi 1 , J Imanifard 1 , Sh Mehrpoor 1 , J Gerdabi 1 , M Karimi 2 , *

AUTHORS INFORMATION

1 Hematology Research Center, Shiraz University of Medical Science, Iran

2 Professor of Pediatric Hematology-Oncology, Hematology Research Center, Nemazee Hospital, Karimim@sums.ac.ir, Iran

How to Cite: Dehghani S, Dashtarzhen A, Nasirabadi S, Dehbozorgian J, Afrasiabi A, et al. Combined ?-thalassemia and Hemoglobin J-Iran (?77 His ? Asp). A Family Study in southern Iran, Iran Red Crescent Med J. Online ahead of Print ; 13(8):586-589.

ARTICLE INFORMATION

Iranian Red Crescent Medical Journal: 13 (8); 586-589
Article Type: Case Report
Received: December 12, 2010
Accepted: April 11, 2011

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Abstract

We report a 23-year-old man and three members of his family with Hb J-Iran confirmed by electrophoresis, chain separation by high performance liquid chromatography and sequencing. Alpha thalassemia was also confirmed in two family members. The substitution at β77 led to a higher negative charge of the βJ-Iran subunit, which enhanced its electrostatic attraction for the normal positively-charged α subunit. Therefore, more Hb J-Iran than Hb A forms in the red blood cells of heterozygotes. In α-thalassemia, the more attractive βJ-Iran subunit out-competes βA subunits in forming assemblies with deficient α subunits, so even more Hb J-Iran was formed.

Keywords

Hb J-Iran ?-thalassemia Hemoglobin variants Electrophoresis High performance liquid chromatography

© 0, Author(s). This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License (http://creativecommons.org/licenses/by-nc/4.0/) which permits copy and redistribute the material just in noncommercial usages, provided the original work is properly cited.

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