The organization of microtubules and microtubule coils in giant platelet disorders.
The organization of microtubules and microtubule coils in giant platelet disorders.
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发表时间：
1984-09
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通讯作者：
J. White;J. Sauk
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作者：
J. White;J. Sauk
正常人的血小板呈盘状。透镜状的外观是由细胞膜下沿最大周长的微管组成的环状带支撑的。来自巨大血小板疾病患者的一些细胞也是盘状的，但大多数巨大血小板是球形的。在本研究中，我们对灰色血小板综合征（GPS）、May-Hegglin异常（MHA）和爱泼斯坦综合征（ES）患者的血小板进行了薄切片和全片阴性染色，并采用微管蛋白单克隆抗体间接免疫荧光法测定其微管系统的组织。许多GPS血小板和一些ES和MHA血小板呈盘状，并含有微管束。带中的线圈数量增加了10-20倍。巨大的球形血小板也含有更多的单个微管和微线圈，但它们没有被组织成圆周束。免疫荧光显示在巨大的细胞中有一个组装的微管蛋白组织，使人联想到纱线球。微管不能组织成圆周带可以解释为什么大多数巨大细胞呈球形。
Normal human platelets are characteristically discoid in shape. The lentiform appearance is supported by a circumferential band of microtubules lying just under the cell membrane along its greatest circumference. Some of the cells from patients with giant platelet disorders are also disk-shaped, but the majority of their huge platelets are spherical. In the present study platelets from patients with the Gray platelet syndrome (GPS), May-Hegglin anomaly (MHA), and Epstein's syndrome (ES) were examined in thin sections and negatively stained whole mounts, and by indirect immunofluorescence with a monoclonal antibody to tubulin for determination of the organization of their microtubule systems. Many GPS platelets and some ES and MHA platelets were discoid and contained circumferential bundles of microtubules. The number of coils in the band was increased 10-20-fold. Giant spherical platelets also contained increased numbers of individual microtubules and coils, but they were not organized into circumferential bundles. Immunofluorescence revealed an organization of assembled tubulin in the huge cells, suggestive of balls of yarn. Failure of the microtubules to organize into a circumferential band may explain why the majority of the huge cells have a spherical form.