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原发性获得性铁粒幼细胞贫血患者环形铁粒幼细胞合成核酸和蛋白质的能力。

Capacity of ringed sideroblasts to synthesize nucleic acids and protein in patients with primary acquired sideroblastic anaemia.

作者信息

Wickramasinghe S N, Hughes M

出版信息

Br J Haematol. 1978 Mar;38(3):345-52. doi: 10.1111/j.1365-2141.1978.tb01053.x.

Abstract

Bone marrow cells from two patients with primary acquired sideroblastic anaemia were studied using the technique of electron microscope autoradiography. Whereas a significant proportion of erythroblasts containing small to moderate quantities of intramitochondrial deposits incorporated [3H]thymidine into DNA, the majority of cells containing large quantities of intramitochondrial deposits did not. These results are consistent with the idea that several of the early polychromatic cells with large quantities of deposits become arrested in their progress through the cell cycle. The data also revealed that the accumulation of increasing quantities of iron-containing material within the mitochondria of an erythroblast was frequently (but not invariably) associated with and was possibly responsible for (I) a depression of RNA synthesis and (2) a more marked depression of protein synthesis. A small proportion of the erythroblasts showing a depression of protein synthesis also displayed various non-specific ultrastructural abnormalities indicative of cellular injury such as the presence of intranuclear clefts, nuclear membrane-associated myelin figures and lipid-laden intracytoplasmic vacuoles.

摘要

运用电子显微镜放射自显影技术,对两名原发性获得性铁粒幼细胞贫血患者的骨髓细胞进行了研究。虽然相当一部分含有少量至中等量线粒体内沉积物的成红细胞能将[³H]胸腺嘧啶核苷掺入DNA,但大多数含有大量线粒体内沉积物的细胞却不能。这些结果与以下观点一致,即一些含有大量沉积物的早期多色性细胞在细胞周期进程中停滞。数据还显示,成红细胞线粒体内含铁物质数量的不断积累经常(但并非总是)与RNA合成的抑制以及更显著的蛋白质合成抑制相关,并且可能是其原因。一小部分显示蛋白质合成受抑制的成红细胞还表现出各种指示细胞损伤的非特异性超微结构异常,如核内裂隙、核膜相关的髓鞘样结构以及充满脂质的胞质内空泡。

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