Association of hemoglobin H disease with Hb J-Iran (beta 77 His----Asp): impact on subunit assembly.
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.
Rahbar
S
S
Department of Hematology and Bone Marrow Transplantation, City of Hope National Medical Center, Duarte, CA 91010-0269.
Bunn
H F
HF
D005914:Globins / Q000235:genetics; D006448:Hemoglobin J; D006455:Hemoglobins, Abnormal; D006801:Humans; D046911:Macromolecular Substances; D013329:Structure-Activity Relationship; D013789:Thalassemia / Q000235:genetics*
D006455:Hemoglobins, Abnormal; D046911:Macromolecular Substances; D006448:Hemoglobin J; C036257:hemoglobin J Iran; D005914:Globins