Burke W G, Chen S H, Scott C R, Ammann A J
J Cell Physiol. 1977 Jul;92(1):109-13. doi: 10.1002/jcp.1040920113.
Cultured skin fibroblasts from a patient with T-cell immune deficiency and an absence of purine nucleoside phosphorylase activity in red cells were assayed for their capacity to metabolize inosine and guanosine. The cultured fibroblasts were lacking activity of nucleoside phosphorylase and, compared to normal fibroblasts, could incorporate only 2% and 4% of 14C-inosine and 3H-guanosine, respectively, into acid precipitable material. Autoradiography visually confirmed the failure of the NP deficient cell line to incorporate the nucleosides into nuclear material. The physiological mechanism by which the deficiency of purine nucleoside phosphorylase causes T-cell dysfunction remains unclear.
对一名患有T细胞免疫缺陷且红细胞中缺乏嘌呤核苷磷酸化酶活性的患者的培养皮肤成纤维细胞进行了检测，以评估其代谢肌苷和鸟苷的能力。培养的成纤维细胞缺乏核苷磷酸化酶活性，与正常成纤维细胞相比，分别只能将14C-肌苷和3H-鸟苷的2%和4%掺入酸沉淀物质中。放射自显影直观地证实了缺乏NP的细胞系无法将核苷掺入核物质中。嘌呤核苷磷酸化酶缺乏导致T细胞功能障碍的生理机制仍不清楚。
