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[血液中异常血红蛋白M导致的遗传性发绀:其检测、鉴定及特性]

[Hereditary cyanosis caused by the presence of abnormal hemoglobin M in the blood: its detection, identification and properties].

作者信息

Kazanets E G, Andreeva A P, Khangulov S V, Tokarev Iu N

出版信息

Gematol Transfuziol. 1990 Mar;35(3):9-13.

PMID:2361589
Abstract

A total of 17 cases of anomalous hemoglobin M (Hb M) were detected among subjects of varying nationalities in different regions of the USSR. The methods used for identification of Hb M Saskatoon, Hb M Boston, Hb M Iwate, Hb M Hyde Park have been described, among them--electron paramagnetic resonance. Spectral characteristics, electrophoretic mobility of these Hb in pH gradient, reaction with cyanides, thermal stability, in vitro reduction with methemoglobin reductase, isolated from donor's red blood cells, have been investigated. The functional parameters (log P50 and n) have been determined for hemolysates containing anomalous hemoglobin, as well as for chromatographically pure fractions of anomalous hemoglobins. The importance of the proper diagnosis of hemoglobinosis M has been stressed.

摘要

在苏联不同地区的不同国籍人群中,共检测出17例异常血红蛋白M(Hb M)。文中描述了用于鉴定Hb M萨斯卡通、Hb M波士顿、Hb M岩手、Hb M海德公园的方法,其中包括电子顺磁共振。研究了这些Hb在pH梯度中的光谱特征、电泳迁移率、与氰化物的反应、热稳定性、用从供体红细胞中分离出的高铁血红蛋白还原酶进行的体外还原。还测定了含有异常血红蛋白的溶血产物以及异常血红蛋白的色谱纯级分的功能参数(log P50和n)。强调了正确诊断血红蛋白病M的重要性。

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