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首页> 美国卫生研究院文献>The Journal of Clinical Investigation >Decreased content and surface expression of alpha-granule membrane protein GMP-140 in one of two types of platelet alpha delta storage pool deficiency.
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Decreased content and surface expression of alpha-granule membrane protein GMP-140 in one of two types of platelet alpha delta storage pool deficiency.

机译：两种类型的血小板α-δ存储池缺乏症之一中α-颗粒膜蛋白GMP-140的含量和表面表达降低。

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摘要

To determine whether alpha-granule membranes are present in platelets of patients with storage pool deficiencies of both alpha and dense granules (alpha delta-SPD), we examined the content and surface expression of the alpha-granule membrane protein GMP-140 in one patient (J.C.) with a severe alpha-granule deficiency and in three members of a family (family C) with milder alpha-granule deficiencies. Surface expression of GMP-140 in stimulated platelets, assessed by flow cytometric measurements of the binding of two anti-GMP-140 monoclonal antibodies, was 24-38% of normal values in platelets from patient J.C., vs. 60-95% of normal values in family C. Total platelet content of GMP-140, determined in platelet lysates by antigen-capture ELISA, was 49% of normal in patient J.C., but normal in the members of family C. Platelets of patient J.C. were found to be heterogeneous with respect to GMP-140 content and surface expression by both flow cytometry and immunogold electron microscopy. Approximately 80% of her platelets expressed little or no GMP-140 after stimulation, whereas the remaining 20% expressed normal amounts of GMP-140 and showed extensive immunogold labeling of typical alpha-granules and clear vacuoles. No such heterogeneity was found in platelets from family C. These findings in the severe alpha delta-SPD patient are in clear contrast to the observations of normal GMP-140 content in the three other alpha delta-SPD patients, and in patients with the gray platelet syndrome, reported previously by others. These results illustrate the phenotypic heterogeneity of alpha-granule deficiencies in human platelets, and suggest that a defect in granule formation in the megakaryocytes may account for the alpha-granule defect in at least one form of alpha delta-SPD.
机译：为了确定具有α和致密颗粒（αδ-SPD）的储库缺陷的患者的血小板中是否存在α颗粒膜，我们检查了一名患者中α颗粒膜蛋白GMP-140的含量和表面表达（JC）患有严重的α-颗粒缺乏症，并且在一个较轻的α-颗粒缺乏症的家庭（C族）的三个成员中。通过流式细胞术测量两种抗GMP-140单克隆抗体的结合来评估刺激的血小板中GMP-140的表面表达，是JC患者血小板正常值的24-38％，而正常人的血小板为60-95％通过抗原捕获ELISA在血小板裂解物中测定的GMP-140的总血小板含量为JC患者正常水平的49％，但在C家庭成员中为正常。发现JC患者血小板异质流式细胞仪和免疫金电子显微镜观察GMP-140含量和表面表达。刺激后约80％的血小板表达很少或不表达GMP-140，而其余20％则表达正常量的GMP-140，并显示出典型α颗粒和透明液泡的广泛免疫金标记。在C家族的血小板中未发现这种异质性。在严重的α-δ-SPD患者中的这些发现与其他三名α-δ-SPD患者以及灰色患者中GMP-140含量正常的观察结果形成鲜明对比血小板综合征，先前已被其他人报道。这些结果说明了人类血小板中α-颗粒缺陷的表型异质性，并表明在巨核细胞中颗粒形成的缺陷可以解释至少一种形式的α-δ-SPD中的α-颗粒缺陷。

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