Author/Authors :
Dehghani, SJ Hematology Research Center - Shiraz University of Medical Science, Shiraz , Amiri Dashtarzhen, A Hematology Research Center - Shiraz University of Medical Science, Shiraz , Nasirabadi, Sh Hematology Research Center - Shiraz University of Medical Science, Shiraz , Dehbozorgian, J Hematology Research Center - Shiraz University of Medical Science, Shiraz , Afrasiabi, AR Hematology Research Center - Shiraz University of Medical Science, Shiraz , Morshedi, N Hematology Research Center - Shiraz University of Medical Science, Shiraz , Imanifard, J Hematology Research Center - Shiraz University of Medical Science, Shiraz , Mehrpoor, Sh Hematology Research Center - Shiraz University of Medical Science, Shiraz , Gerdabi, J Hematology Research Center - Shiraz University of Medical Science, Shiraz , Karimi, M Hematology Research Center - Shiraz University of Medical Science, Shiraz
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 outcompetes
βA subunits in forming assemblies with deficient α subunits, so even more Hb J-Iran was formed.
Keywords :
Hb J-Iran , α-thalassemia , Hemoglobin variants , Electrophoresis , High performance liquid chromatography
