一些具有改变的氧亲和力的罕见血红蛋白变体；林雪平血红蛋白[β36（C2）脯氨酸→苏氨酸]、加勒比血红蛋白[β91（F7）亮氨酸→精氨酸]和桑尼布鲁克血红蛋白[β36（C2）脯氨酸→精氨酸]。

Some rare hemoglobin variants with altered oxygen affinities; Hb linkoping [beta 36(C2)Pro----Thr], Hb Caribbean [beta 91(F7)Leu----Arg], and Hb Sunnybrook [beta 36(C2)Pro----Arg].

作者信息

Ali M A, Pinkerton P, Chow S W, Zaetz S D, Wilson J B, Webber B B, Hu H, Kutlar A, Kutlar F, Huisman T H

机构信息

Hemoglobinopathy Reference Laboratory, St. Joseph's Hospital, Hamilton, Ontario, Canada.

出版信息

Hemoglobin. 1988;12(2):137-48. doi: 10.3109/03630268808998020.

DOI:10.3109/03630268808998020

PMID:3384706

Abstract

A few rare hemoglobin variants with altered functional properties have been observed in Canadian subjects with either an erythrocytosis or mild anemia. These variants were Hb Alberta [beta 101(G3)Glu----Gly], Hb Linkoping [beta 36(C2)Pro----Thr], a new variant Hb Sunnybrook [beta 36(C2)Pro----Arg], and Hb Caribbean [beta 91(F7)Leu----Arg]. Short clinical descriptions of the subjects are given, the characterization of the variants is described in detail (except for Hb Alberta), while data from some functional analyses are provided. Comparisons with previously published data have been made and the unusual chromatographic property of two abnormal beta chains in a reversed phase high performance liquid chromatographic system is reviewed.

摘要

在患有红细胞增多症或轻度贫血的加拿大受试者中，已观察到一些具有改变功能特性的罕见血红蛋白变体。这些变体包括阿尔伯塔血红蛋白[β101(G3)谷氨酸→甘氨酸]、林雪平血红蛋白[β36(C2)脯氨酸→苏氨酸]、一种新变体森尼布鲁克血红蛋白[β36(C2)脯氨酸→精氨酸]和加勒比血红蛋白[β91(F7)亮氨酸→精氨酸]。给出了受试者的简短临床描述，详细描述了变体的特征（阿尔伯塔血红蛋白除外），同时提供了一些功能分析的数据。已与先前发表的数据进行了比较，并回顾了反相高效液相色谱系统中两条异常β链的异常色谱特性。