镰仓血红蛋白[α40（C5）赖氨酸→甲硫氨酸]：一种具有增加氧亲和力的新型α链变体。

Hb Kanagawa [alpha 40(C5)Lys----Met]: a new alpha chain variant with an increased oxygen affinity.

作者信息

Miyashita H, Hashimoto K, Mohri H, Ohokubo T, Harano T, Harano K, Imai K

机构信息

First Department of Internal Medicine, Yokohama City University School of Medicine, Japan.

出版信息

Hemoglobin. 1992;16(1-2):1-10. doi: 10.3109/03630269209005670.

DOI:10.3109/03630269209005670

PMID:1634355

Abstract

An abnormal hemoglobin was suspected in a 70-year-old Japanese male with cerebral infarction and erythremia with high performance liquid chromatography assay of Hb A1c. The hemoglobin variant migrated to the anode more rapidly than Hb A. Structure determination studies, including amino acid analysis of the abnormal peptide and DNA sequencing of a partially cloned alpha-globin gene, demonstrated that it is a new hemoglobin variant which has been named Hb Kanagawa [alpha 40(C5)Lys----Met]. This variant showed an increased oxygen affinity, decreased heme-heme interaction, and a lowered 2,3-diphosphoglycerate effect relative to normal.

摘要

一名70岁的日本男性患有脑梗死和红细胞增多症，通过高效液相色谱法检测糖化血红蛋白时，怀疑存在异常血红蛋白。该血红蛋白变体比血红蛋白A向阳极迁移得更快。结构测定研究，包括对异常肽段的氨基酸分析和部分克隆的α-珠蛋白基因的DNA测序，表明它是一种新的血红蛋白变体，已被命名为血红蛋白镰仓[α40(C5)赖氨酸→甲硫氨酸]。相对于正常情况，该变体显示出氧亲和力增加、血红素-血红素相互作用降低以及2,3-二磷酸甘油酸效应降低。