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慢性巨核细胞-粒细胞性骨髓增生症中正常及病理性巨核细胞系的冷冻断裂

Freeze-fracture of the normal and pathological megakaryocyte lineage in chronic megakaryocytic-granulocytic myelosis.

作者信息

Thiele J, Ballard A C, Georgii A

出版信息

Virchows Arch B Cell Pathol. 1977 Jan 20;23(1):33-51. doi: 10.1007/BF02889118.

Abstract

Freeze-fracture and thin sections were performed on human bone marrow of chronic megakaryocytic-granulocytic myelosis (CMGM) to study the three-dimensional fine structure and maturation of normal and atypical megakaryocytes and thrombocytes. In the many normally maturing megakaryocytes the development of the demarcation membrane system (DMS) was best investigated by comparison of thin sections with freeze-fracture replicas. The DMS shows no connections with the Golgi apparatus or rough-surfaced endoplasmic reticulum, but originates from tubular infoldings of the plasma membrane. These infoldings are always in continuity with the extracellular space and form an intracellular membranous pool by branching and coalescing of flattened tubules from which finally the perforated cisternae of the DMS arise. Freeze-fracture of the normal thrombocytes confirms earlier findings. The abnormal giant platelets seen in CMGM display extensive areas of smooth membranes of a spongy structure consisting of dense tubules surrounded by the labyrinth of the surface-connected system. Their physiological significance in these atypical platelets remains unsolved.

摘要

对慢性巨核细胞-粒细胞性骨髓增生症(CMGM)患者的人骨髓进行冷冻断裂和薄片制作,以研究正常和非典型巨核细胞及血小板的三维精细结构和成熟过程。在许多正常成熟的巨核细胞中,通过将薄片与冷冻断裂复制品进行比较,能最好地研究分界膜系统(DMS)的发育情况。DMS与高尔基体或糙面内质网无连接,而是起源于质膜的管状内褶。这些内褶始终与细胞外空间相连,并通过扁平小管的分支和融合形成细胞内的膜池,最终由此产生DMS的穿孔池。正常血小板的冷冻断裂证实了早期的研究结果。在CMGM中看到的异常巨大血小板显示出广泛的海绵状结构的光滑膜区域,该结构由密集的小管组成,周围是表面连接系统的迷宫。它们在这些非典型血小板中的生理意义仍未解决。

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