van der Weyden M B, Bailey L
Clin Chim Acta. 1978 Jan 2;82(1-2):179-84. doi: 10.1016/0009-8981(78)90041-4.
A radiochromatographic method is described for measuring adenosine deaminase and purine nucleoside phosphorylase activity in cells from human peripheral blood. The respective substrates, [8-14C]adenosine or [8-14C]inosine, are converted either to inosine and hypoxanthine or hypoxanthine, respectively. A single simple and rapid chromatographic procedure is used to isolate the products of both reactions. The mean normal activity (nmol h-1mg-1) of ADA for erythrocytes is 63 +/- 24 (+/- 1 S.D.) for leukocytes, 750 +/- 280 and for lymphocytes, 2105 +/- 1170. Corresponding activities for purine nucleoside phosphorylase are 1850 +/- 490, 3665 +/- 1170 and 5890 +/- 2030. With the described methods a further patient with severe combined immuno-deficiency and adenosine deaminase deficiency has been identified.
本文描述了一种放射色谱法,用于测量人外周血细胞中的腺苷脱氨酶和嘌呤核苷磷酸化酶活性。各自的底物,即[8-14C]腺苷或[8-14C]肌苷,分别转化为肌苷和次黄嘌呤或次黄嘌呤。采用单一简单快速的色谱程序分离两个反应的产物。红细胞中ADA的平均正常活性(nmol h-1mg-1)为63±24(±1标准差),白细胞为750±280,淋巴细胞为2105±1170。嘌呤核苷磷酸化酶的相应活性分别为1850±490、3665±1170和5890±2030。通过所述方法,又发现了一名患有严重联合免疫缺陷和腺苷脱氨酶缺乏症的患者。