杜氏肌营养不良症（DMD）患者及杜氏肌营养不良症携带者（智人）红细胞碱性磷酸酶的变构转变

Allosteric transition of erythrocyte alkaline phosphatase from Duchenne muscular dystrophy (DMD) patients and Duchenne muscular dystrophy carriers (Homo sapiens).

Goldemberg A L, García A M, Fernández H, Fortunato M, Sánchez J J, Trucco R E

Facultad de Ciencias Exactas y Naturales, U.N. de Mar del Plata, Argentina.

Int J Biochem. 1988;20(7):703-6. doi: 10.1016/0020-711x(88)90165-6.

Abstract

The kinetic properties of the p-nitrophenylphosphatase (EC 3.1.3.1) from erythrocytes was investigated in DMD-patients and DMD-carriers. 2. A different allosteric behaviour in the p-nitrophenylphosphatase from DMD-patients and DMD-carriers compared to controls is supported by the following findings: (a) values of n altered in F- inhibition of (K+)-activated p-nitrophenylphosphatase with Hill coefficients -1.5, -2.2 and -3.1; (b) heterotropic effect of increased concentration of Mg2+ on F- inhibition which is reverted by K+ in DMD-carriers and in control, but not in DMD-patients. 3. Evidence is presented showing that in DMD-patients and in DMD-carriers the interaction membrane-enzyme is different from the corresponding controls.

摘要

对杜氏肌营养不良症（DMD）患者和DMD基因携带者红细胞中的对硝基苯磷酸酶（EC 3.1.3.1）的动力学特性进行了研究。2. 以下研究结果支持了DMD患者和DMD基因携带者的对硝基苯磷酸酶与对照组相比具有不同变构行为的观点：（a）在氟抑制（钾离子）激活的对硝基苯磷酸酶时，n值发生改变，希尔系数分别为-1.5、-2.2和-3.1；（b）镁离子浓度增加对氟抑制的异促效应，在DMD基因携带者和对照组中，钾离子可使其逆转，但在DMD患者中则不然。3. 有证据表明，DMD患者和DMD基因携带者中膜 - 酶的相互作用与相应对照组不同。