Literature DB >> 3676514

Association of hemoglobin H disease with Hb J-Iran (beta 77 His----Asp): impact on subunit assembly.

S Rahbar1, H F Bunn.   

Abstract

A young Iranian female was found to be heterozygous for hemoglobin (Hb) J-Iran (beta 77 His----Asp) in combination with Hb H disease. The proportion of Hb J in the patient's hemolysate was surprisingly high: 65% Hb J, 30% Hb A. Thus, the interaction of a negatively charged beta subunit variant of Hb with alpha-thalassemia leads to a marked increase in the relative amount of the variant Hb within red cells. This observation provides further support for an electrostatic model of Hb subunit assembly.

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Year:  1987        PMID： 3676514

Source DB:  PubMed          Journal:  Blood        ISSN： 0006-4971            Impact factor:   22.113


  2 in total

1.  Hb Costa Rica or alpha 2 beta 2 77(EF1)His --> Arg: the first example of a somatic cell mutation in a globin gene.

Authors:  W E Rodriguez Romero; M Castillo; M A Chaves; G F Saenz; L H Gu; J B Wilson; E Baysal; N S Smetanina; J Y Leonova; T H Huisman
Journal:  Hum Genet       Date:  1996-06       Impact factor: 4.132

2.  Combined α-thalassemia and Hemoglobin J-Iran (β77 His → Asp). A Family Study in southern Iran.

Authors:  S J Dehghani; A Amiri Dashtarzhen; Sh Nasirabadi; J Dehbozorgian; A R Afrasiabi; N Morshedi; J Imanifard; Sh Mehrpoor; J Gerdabi; M Karimi
Journal:  Iran Red Crescent Med J       Date:  2011-08-01       Impact factor: 0.611
  2 in total

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