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患有丙酮酸激酶缺乏症的巴仙吉犬红细胞丙酮酸激酶的电泳、免疫学及动力学特征

Electrophoretic, immunologic and kinetic characterization of erythrocyte pyruvate kinase in the Basenji dog with pyruvate kinase deficiency.

作者信息

Nakashima K, Miwa S, Shinohara K, Oda E, Tajiri M

出版信息

Tohoku J Exp Med. 1975 Oct;117(2):179-85. doi: 10.1620/tjem.117.179.

Abstract

The electrophoretic mobility and the immunologic specificity of erythrocyte pyruvate kinase (PK) of the homozygous Basenji dog with PK deficiency were identical to those of normal M2-type PK isozyme seen in the white cell but not to those of the erythrocyte PK isozyme. Kinetic properties and stability were also consistent with the M2-type PK isozyme. Defective PK in the homozygous red cell was due to the absence of the erythrocyte PK isozyme and the compensatory presence of M2-type PK isozyme, as seen in the severe classical type PK deficiency in man.

摘要

患有丙酮酸激酶(PK)缺乏症的纯合巴辛吉犬红细胞丙酮酸激酶的电泳迁移率和免疫特异性与在白细胞中所见的正常M2型PK同工酶相同,但与红细胞PK同工酶不同。动力学性质和稳定性也与M2型PK同工酶一致。纯合红细胞中PK缺陷是由于缺乏红细胞PK同工酶以及M2型PK同工酶的代偿性存在,这与人类严重经典型PK缺乏症所见情况相同。

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