Johnson S M, North M E, Asherson G L, Allsop J, Watts R W, Webster A D
Lancet. 1977 Jan 22;1(8004):168-70. doi: 10.1016/s0140-6736(77)91765-2.
The mean activity of purine 5'-nucleotidase (E.C.3.1.3.5) in lymphocytes was significantly lower in patients with non-familial adult onset "variable" primary hypogammaglobulinaemia than in non-hypogammaglobulinaemic control subjects. These findings suggest that 5'-nucleotidase activity is necessary for normal lymphocyte function. This may be related to its role in facilitating the transfer of newly formed purines across cell membranes by converting them from nucleotides to nucleosides. This enzyme defect may be a characteristic metabolic lesion in at least some of the patients; alternatively, it may be secondary to some other metabolic defect or plasma-membrane change.
非家族性成人起病“可变型”原发性低丙种球蛋白血症患者淋巴细胞中嘌呤5'-核苷酸酶(E.C.3.1.3.5)的平均活性显著低于非低丙种球蛋白血症对照受试者。这些发现表明5'-核苷酸酶活性对于正常淋巴细胞功能是必需的。这可能与其通过将新形成的嘌呤从核苷酸转化为核苷来促进其跨细胞膜转运的作用有关。这种酶缺陷可能是至少部分患者的特征性代谢病变;或者,它可能继发于其他一些代谢缺陷或质膜变化。