Dmitrieva M G, Kazanets E G, Molchanova T P, Karpova I V, Sevast'ianova M G, Andreeva A P
Gematol Transfuziol. 1991 Jul;36(7):9-11.
Hemoglobin M-Boston comprising 30% of the total blood Hb was detected in two members of a Russian family. The stages of its identification were described. An analysis was made of probands' whole blood oxy-Hb dissociation curves. At pO2 = 100 mm Hg oxygenation of Hb M-Boston beta-chains was 44% only, while mutant alpha-chains were completely oxidated. Despite a significant decrease in arterial blood oxygen content arteriovenous variation by O2 content at pO2 differential from 100 to 40 mm Hg is within the normal level due to various directions of changes in P50 and Hill's coefficient of probands' whole blood. The authors consider that pronounced cyanosis in Hb M-Boston carriers is not the consequence of tissue hypoxia but is caused by a higher content of nonfunctioning Hb M-Boston that changes the blood color.
在一个俄罗斯家庭的两名成员中检测到占总血红红蛋白30%的血红蛋白M-波士顿。描述了其鉴定阶段。对先证者全血氧合血红蛋白解离曲线进行了分析。在pO2 = 100 mmHg时，血红蛋白M-波士顿β链的氧合仅为44%，而突变的α链则完全被氧化。尽管动脉血氧含量显著降低，但由于先证者全血的P50和希尔系数变化方向不同，在pO2从100 mmHg降至40 mmHg时，氧含量的动静脉变化仍在正常范围内。作者认为，血红蛋白M-波士顿携带者明显的发绀不是组织缺氧的结果，而是由改变血液颜色的无功能血红蛋白M-波士顿含量较高所致。
