丙酮酸激酶与“高ATP综合征”

Pyruvate kinase and the "high ATP syndrome".

作者信息

Staal G E, Jansen G, Roos D

出版信息

J Clin Invest. 1984 Jul;74(1):231-5. doi: 10.1172/JCI111406.

DOI:10.1172/JCI111406

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC425205/

Abstract

The erythrocytes of a patient with the so-called "high ATP syndrome" were characterized by a high ATP content and low 2,3-diphosphoglycerate level. The pyruvate kinase activity was specifically increased (about twice the normal level). After separation of the erythrocytes according to age by discontinuous Percoll density centrifugation, the pyruvate kinase activity was found to be increased in all Percoll fractions. Pyruvate kinase of the patient's cells was characterized by a decreased K0.5 for the substrate phosphoenolpyruvate and no inhibition by ATP. The Michaelis constant (Km) value for ADP, the nucleotide specificity, the thermostability, pH optimum, and immunological specific activity were normal. It is concluded that the high pyruvate kinase activity is due to a shift in the R(elaxed) in equilibrium T(ight) equilibrium to the R(elaxed) form.

摘要

患有所谓“高ATP综合征”患者的红细胞具有ATP含量高和2,3 - 二磷酸甘油酸水平低的特征。丙酮酸激酶活性特异性增加（约为正常水平的两倍）。通过不连续Percoll密度离心按年龄分离红细胞后，发现所有Percoll组分中的丙酮酸激酶活性均增加。患者细胞的丙酮酸激酶对底物磷酸烯醇丙酮酸的K0.5降低，且不受ATP抑制。ADP的米氏常数（Km）值、核苷酸特异性、热稳定性、最适pH和免疫比活性均正常。结论是丙酮酸激酶活性高是由于平衡态从T（紧密）态向R（松弛）态转变所致。

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