我国异常血红蛋白的研究3例Hb J Bangkok及其一级结构分析

Investigation of Abnormal Hemoglobin in China——Analysis of 3 Cases of HbJ Bangkok and Its Primary Structure

1981年本文部分作者在陕西商洛地区普查血红蛋白病时,在两个无关家系中发现一种泳动速度相同的快速血红蛋白,其电泳位置相当于J组,同年,又在福建龙岩地区发现一家系与上述相同之快速Hb。

During 1980s, in a general survey of hemoglobinopathy in 18 provinces, two cases of hemoglobin variants—Hb J were found in two unrelated families in Shang Xian of Shaanxi province. The electrophoretic mobility of these two cases were the same. At the same time, a similar case with fast mobility was found in Long Yan district ofFujian province. The 3 proposites of this hemoglobinopathy were all Han race. They all are 11 years old, two of them are girls, and the other one is a boy. pedigree study showed that the mode of inheritance were in consistent with autosomal co-dominant. They have no clinical symptoms.The result of the structural analysis, demonstrated that all three cases are Hb J Bangkok β56(D7) Gly-Asp.From our study, it signified that the distribution of Hb J Bangkok β56(D7) Gly-Asp is not limited in south part of China, but also spreaded to North part of China.