PROTEIN-SYNTHESIS AND STORAGE IN HUMAN-PLATELETS - A DEFECTIVE STORAGE OF FIBRINOGEN IN PLATELETS IN GLANZMANNS-THROMBASTHENIA
PROTEIN-SYNTHESIS AND STORAGE IN HUMAN-PLATELETS - A DEFECTIVE STORAGE OF FIBRINOGEN IN PLATELETS IN GLANZMANNS-THROMBASTHENIA
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DOI：
10.1016/0304-4165(87)90112-7
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发表时间：
1987-08-13
期刊：
影响因子：
--
通讯作者：
NURDEN, AT
中科院分区：
文献类型：
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作者：
BELLOC, F;HEILMANN, E;NURDEN, AT
进行体内代谢标记实验以研究人血小板合成和储存血小板生成素和血小板反应蛋白能力。通过SDS-聚丙烯酰胺凝胶电泳分析新合成的蛋白质。结果进行了比较与血小板与Glanzmann的血栓无力症患者的内源性纤维蛋白原水平严重降低。正常人血小板能够合成纤维蛋白原和血小板反应蛋白的不同亚基，并将它们组装成天然纤维蛋白原和血小板反应蛋白分子。该合成被放线菌酮抑制。在Glanzmann's血小板无力症患者的血小板中观察到纤维蛋白原和血小板反应素的合成。然而，放射性标记的纤维蛋白原不再检测后，18小时的非放射性追踪，虽然它保留在控制血小板。在同一追踪期内，患者的新合成血小板反应蛋白正常保存。当研究放射性纤维蛋白原的命运时，发现其在Glanzmann血栓无力症血小板中的降解程度与新合成的细胞质蛋白相同，而在对照血小板中发生的降解较少。我们的结论是，人类血小板保持剩余的能力，合成纤维蛋白原和缺乏Glanzmann的血小板无力症的结果从存储异常，而不是从合成缺陷。
In vivo metabolic labelling experiments were performed to investigate that ability of human platelets to synthesize and store fibringen and thrombospondin. Newly synthesized proteins were analyzed by SDS-polyacrylamide gel electrophoresis. Results were compared with those obtained for the platelets of a patient with Glanzmann''s thrombasthenia where endogenous fibrinogen levels were severely reduced. Normal human platelets were able to synthesize the different subunits of fibrinogen and thrombospondin and to assemble them into native fibrinogen and thrombospondin molecules. This synthesis was inhibited by cycloheximide. Synthesis of both fibrinogen and thrombosponidn was observed in the platelets of the Glanzmann''s thrombasthenia patient. However, radiolabelled fibrinogen was no longer detected after an 18-h non-radioactive chase, although it was retained in the control platelets. Neosynthesized thrombospondin of the patient was normally preserved during the same chase period. When the fate of the radioactive fibrinogen was studied, it was found to be degraded in Glanzmann''s thrombasthenia platelets to the same extent as neosynthesized cytoplasmic proteins, whereas in control platelets less degradation had occurred. We conclude that human platelets maintain a residual capacity to synthesize fibrinogen and that its deficiency in Glanzmann''s thrombasthenia results from a storage abnormality and not from a synthesis defect.