戈登堡血红蛋白或α2β2 145位酪氨酸被天冬氨酸取代，一种新的高氧亲和力血红蛋白变体。

Hemoglobin Fort Gordon or alpha2beta2145 Tyr replaced by Asp, a new high-oxygen-affinity hemoglobin variant.

作者信息

Kleckner H B, Wilson J B, Lindeman J G, Stevens P D, Niazi G, Hunter E, Chen C J, Huisman T H

出版信息

Biochim Biophys Acta. 1975 Aug 19;400(2):343-7. doi: 10.1016/0005-2795(75)90189-0.

Abstract

Hemoglobin Fort Gordon, alpha2beta2145 Tyr replaced by Asp (HC2), has been observed in a 20-year-old black male with compensatory erythrocytosis. The variant was readily identified by electrophoresis and chromatography, and comprised about 30% of the red cell hemoglobin. The substitution was identified through analyses of tryptic peptides of various digests of the isolated beta chain. The oxygen affinity of whole blood was increased; two components were observed one of which had a greatly increased affinity for oxygen and a markedly reduced subunit cooperativity. It appears that the Tyr replaced by Asp substitution resembles the Tyr replaced by His substitution in hemoglobin Bethesda (Bunn, H. F. et al. (1972) J. Clin. Invest. 51, 2299-2309; Olson, J. S. and Gibson, G. H. (1972) J Biol. Chem. 247, 3662-3670; Adamson et al. (1972) J. Clin. Invest. 51, 2883-2888) in that both inhibit the quarternary change of the oxy to the deoxy conformation, resulting in greatly altered functional properties. Studies of a few members of the family were negative.

摘要

在一名患有代偿性红细胞增多症的20岁黑人男性中，发现了血红蛋白戈登堡（α2β2 145位酪氨酸被天冬氨酸取代（HC2））。该变体通过电泳和色谱法很容易被鉴定出来，约占红细胞血红蛋白的30%。通过对分离出的β链各种消化产物的胰蛋白酶肽进行分析，确定了这种取代。全血的氧亲和力增加；观察到两个组分，其中一个对氧的亲和力大大增加，亚基协同性明显降低。似乎酪氨酸被天冬氨酸取代类似于血红蛋白贝塞斯达中酪氨酸被组氨酸取代（邦恩，H.F.等人（1972年）《临床研究杂志》51卷，2299 - 2309页；奥尔森，J.S.和吉布森，G.H.（1972年）《生物化学杂志》247卷，3662 - 3670页；亚当森等人（1972年）《临床研究杂志》51卷，2883 - 2888页），因为两者都抑制了氧合构象向脱氧构象的四级变化，导致功能特性发生很大改变。对该家族的几名成员进行研究结果为阴性。