Rahbar S, Bunn H F
Department of Hematology and Bone Marrow Transplantation, City of Hope National Medical Center, Duarte, CA 91010-0269.
Blood. 1987 Dec;70(6):1790-1.
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.
一名年轻的伊朗女性被发现血红蛋白（Hb）J-伊朗（β77组氨酸→天冬氨酸）杂合子合并Hb H病。患者溶血产物中Hb J的比例出奇地高：65%为Hb J，30%为Hb A。因此，带负电荷的Hbβ亚基变体与α地中海贫血的相互作用导致红细胞内变体Hb的相对量显著增加。这一观察结果为Hb亚基组装的静电模型提供了进一步支持。
