Zerez C R, Lachant N A, Tanaka K R
Enzyme. 1985;34(2):94-7. doi: 10.1159/000469368.
Erythrocytes from patients with hereditary pyrimidine 5'-nucleotidase (P5N, EC 3.1.3.5) deficiency accumulate large quantities of several pyrimidine nucleotides and their derivatives. In addition, the reduced glutathione (GSH) concentration is elevated in erythrocytes from patients with this enzyme deficiency. In the present study, we were unable to demonstrate any effect of various pyrimidine nucleotides and their derivatives on enzymes of glutathione biosynthesis and metabolism. Thus, elevation of GSH levels in erythrocytes from P5N patients is not a result of modulation of these enzymes by pyrimidine nucleotides and their derivatives.
患有遗传性嘧啶5'-核苷酸酶(P5N,EC 3.1.3.5)缺乏症患者的红细胞会积累大量几种嘧啶核苷酸及其衍生物。此外,这种酶缺乏症患者的红细胞中还原型谷胱甘肽(GSH)浓度会升高。在本研究中,我们未能证明各种嘧啶核苷酸及其衍生物对谷胱甘肽生物合成和代谢酶有任何影响。因此,P5N患者红细胞中GSH水平的升高并非嘧啶核苷酸及其衍生物对这些酶进行调节的结果。