Adenosine transport in peripheral blood lymphocytes from Lesch-Nyhan patients.
Adenosine transport in peripheral blood lymphocytes from Lesch-Nyhan patients.
复制标题
Lesch-Nyhan 患者外周血淋巴细胞中的腺苷转运。
作者：
R. Torres;I. Deantonio;C. Prior;J. Puig
我们假设腺苷功能可能与Lesch-Nyhan综合征的某些神经学特征有关，因此，我们对Lesch-Nyhan综合征患者（PBL（LN））和对照组（PBL（C））外周血淋巴细胞中的腺苷转运进行了表征。与PBL（C）相比，PBL（LN）中的腺苷转运显著较低，并且与PBL（C）相比，PBL（LN）中每个细胞定量的[（3）H]硝基苄基硫代肌苷结合的高亲和力位点（B（max））的数量显著较低。与25 μ M次黄嘌呤孵育后，PBL（LN）中的腺苷转运相对于PBL（C）显著降低。与基础条件相比，次黄嘌呤孵育降低PBL（C）中[（3）H]硝基苄基硫代肌苷的结合，但不影响PBL（LN）中的结合。这表明，次黄嘌呤影响腺苷转运控制和次黄嘌呤-鸟嘌呤磷酸核糖转移酶缺陷的细胞通过不同的机制。
We postulated that adenosine function could be related to some of the neurological features of Lesch-Nyhan syndrome and therefore characterized adenosine transport in PBLs (peripheral blood lymphocytes) obtained from Lesch-Nyhan patients (PBL(LN)) and from controls (PBL(C)). Adenosine transport was significantly lower in PBL(LN) when compared with that in PBL(C) and a significantly lower number of high affinity sites for [(3)H]nitrobenzylthioinosine binding were quantified per cell ( B (max)) in PBL(LN) when compared with that in PBL(C). After incubation with 25 microM hypoxanthine, adenosine transport was significantly decreased in PBL(LN) with respect to PBL(C). Hypoxanthine incubation lowers [(3)H]nitrobenzylthioinosine binding in PBL(C), with respect to basal conditions, but does not affect it in PBL(LN). This indicates that hypoxanthine affects adenosine transport in control and hypoxanthine-guanine phosphoribosyltransferase-deficient cells by different mechanisms.