与血红蛋白亚特兰大β75（E19）亮氨酸→脯氨酸突变相关的β141亮氨酸的翻译后修饰。
Posttranslational modification of beta 141 Leu associated with the beta 75(E19)Leu-->Pro mutation in Hb Atlanta.
作者信息
Brennan S O, Shaw J G, George P M, Huisman T H
机构信息
Pathology Department, Christchurch Hospital, New Zealand.
出版信息
Hemoglobin. 1993 Feb;17(1):1-7. doi: 10.3109/03630269308998880.
The recent report of the oxidation of beta 141 Leu in a New Zealand family with Hb Atlanta [ beta 75(E19)Leu-->Pro] prompted us to reinvestigate the original Hb Atlanta case from Georgia. Tryptic peptide maps showed that the modified beta CoT-14 peptide was present together with beta AT-14 in isopropanol precipitates of Hb Atlanta. Amino acid analysis confirmed that beta CoT-14 lacked leucine and mass spectrometry indicated that it had an increased mass of 16 Daltons. These findings support the proposition that the beta 75 Leu-->Pro substitution in the E helix is the direct cause of the beta 141 Leu oxidation.
最近有报道称，在一个患有亚特兰大血红蛋白病[β75(E19)亮氨酸→脯氨酸]的新西兰家庭中，β141亮氨酸发生了氧化，这促使我们重新调查来自佐治亚州的首例亚特兰大血红蛋白病病例。胰蛋白酶肽图显示，在亚特兰大血红蛋白的异丙醇沉淀中，修饰后的β CoT - 14肽与β AT - 14同时存在。氨基酸分析证实β CoT - 14缺乏亮氨酸，质谱分析表明其分子量增加了16道尔顿。这些发现支持了E螺旋中β75亮氨酸→脯氨酸取代是β141亮氨酸氧化的直接原因这一观点。
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