Benke P J, Dittmar D
Pediatr Res. 1976 Jul;10(7):642-6. doi: 10.1203/00006450-197607000-00002.
Conversion of adenosine to inosine is decreased in adenosine deaminase (ADA)-deficient fibroblasts at all concentrations of adenosine tested. Adenosine is not differentially toxic to ADA-deficient fibroblasts except at very high (5 X 10(-4) -1 X 10(-3) M) adenosine levels. Conversion of [14C] adenosine to GTP is not decreased in ADA-deficient cells compared with control cell strains. Adenosine conversion to ATP is the same as that in mutant cells except at high nonphysiologic concentrations, at which it is slightly decreased in ADA-deficient fibroblasts. This effect is probably not related to the biochemical pathology of ADA-deficient lymphocytes in vivo. Uridine, a pyrimidine compound, "rescues" control cells from the effects of adenosine toxicity, as previously reported, but it has no protective effect on ADA-deficient fibroblasts. This suggests that uridine will have no therapeutic role in the treatment of the ADA-deficient form of severe combined immunodeficiency (SCID) disease.
在所有测试的腺苷浓度下，腺苷脱氨酶（ADA）缺陷的成纤维细胞中腺苷向肌苷的转化均减少。除了在非常高的（5×10⁻⁴ - 1×10⁻³ M）腺苷水平外，腺苷对ADA缺陷的成纤维细胞没有差异毒性。与对照细胞系相比，ADA缺陷细胞中[¹⁴C]腺苷向GTP的转化没有减少。除了在高的非生理浓度下，腺苷向ATP的转化与突变细胞中的相同，在高非生理浓度下，ADA缺陷的成纤维细胞中该转化略有减少。这种效应可能与体内ADA缺陷淋巴细胞的生化病理无关。如先前报道的，嘧啶化合物尿苷可使对照细胞免受腺苷毒性的影响，但它对ADA缺陷的成纤维细胞没有保护作用。这表明尿苷在治疗ADA缺陷型严重联合免疫缺陷（SCID）疾病中没有治疗作用。
