Further evidence for a deficient storage pool of adenine nucleotides in platelets from some patients with thrombocytopathia--"storage pool disease".
Further evidence for a deficient storage pool of adenine nucleotides in platelets from some patients with thrombocytopathia--"storage pool disease".
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进一步证据表明，一些患有血小板病的患者的血小板中存在腺嘌呤核苷酸储存池缺陷——“储存池疾病”。
作者：
H. Holmsen;H. Holmsen;H. J. Weiss
对1例血小板减少症患者和6例出血障碍患者的血小板中腺嘌呤核苷酸的代谢进行了研究，这些患者的出血障碍是由于胶原诱导的血小板聚集缺陷与血小板ADP(血小板病态反应)的释放障碍有关。在其中两名患者中，没有发现可能解释释放反应缺陷(血小板病态B)的特殊异常。另4例(血小板增多症A)患者血小板ATP、ADP含量显著降低，ATP/ADP比值升高。血小板与~3H-腺嘌呤孵育后，发现ATP和更引人注目的ADP的比放射性都显著高于正常。这表明，患者的血小板缺乏存在于特殊细胞内颗粒中的非代谢腺嘌呤核苷酸池，这些核苷酸在释放反应中被特异性地从血小板中挤出。在这些患者中，有三名患者的血小板5-羟色胺水平较低，这表明他们的血小板可能缺乏通常在这些颗粒中发现的物质的全部含量。在所有4例血小板增多症A患者中，血小板腺嘌呤摄取正常，但在3例5-羟色胺水平低的患者中，发现静息细胞形成次黄嘌呤的增加。在所研究的所有患者中，ATP在释放反应中分解为肌苷单磷酸酯和次黄嘌呤是正常的。血小板减少症患者的血小板在所有方面都是正常的。
The metalobism of adenine nucleotides in platelets was studied in one patient with thrombasthenia and in six patients whose bleeding disorder has been attributed to a defect in collagen-induced platelet aggregation associated with impaired release of platelet ADP (thrombocytopathia). In two of these patients no specific abnormality was found that might account for the defect in the release reaction (thrombocytopathia B). In the other four patients (thrombocytopathia A), significantly decreased amounts of platelet ATP and ADP and an increase in the ATP/ADP ratio were obtained. The specific radioactivity of both ATP and, more strikingly, ADP that was found after incubating their platelets with 3H-adenine was significantly greater than normal. This indicated that the patients’ platelets lacked the nonmetabolic pool of adenine nucleotides present in specialized intracellular granules and that are specifically extruded from the platelet during the release reaction. Low platelet serotonin values were found in three of these patients, indicating that their platelets may lack the entire content of substances normally found in these granules. In all four of the patients with thrombocytopathia A, for which the name "storage pool disease" is proposed, platelet adenine uptake was normal, but increased hypoxanthine formation by resting cells was found in the three patients with low serotonin values. The breakdown of the ATP to inosine monophopshate and hypoxanthine during the release reaction was normal in all patients studied. Platelets from the patient with thrombasthenia were normal in all respects studied.