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去铁胺对范可尼贫血中染色体不稳定性的部分纠正作用

Partial correction of chromosome instability in Fanconi anemia by desferrioxamine.

作者信息

Porfirio B, Ambroso G, Giannella G, Isacchi G, Dallapiccola B

机构信息

Department of Public Health and Cell Biology, 2nd University of Rome, Italy.

出版信息

Hum Genet. 1989 Aug;83(1):49-51. doi: 10.1007/BF00274146.

Abstract

The action of the iron chelator desferrioxamine (DFO) on the cytogenetic pattern of cultured lymphocytes from Fanconi anemia (FA) patients was investigated. The addition of 10(-4) M DFO throughout the culture time resulted in a 50% reduction of the spontaneous chromosome breakage of FA cells. In addition, the clastogenic action of diepoxybutane on FA lymphocytes was also partly counteracted by DFO. The above findings support the assumption that one of the mechanisms involved in the pathogenesis of FA might be an impaired capacity of the cells from such patients to remove active oxygen species. The relationship between intraleukocyte chelatable iron pool and free radical formation in FA subjects is discussed.

摘要

研究了铁螯合剂去铁胺(DFO)对范可尼贫血(FA)患者培养淋巴细胞细胞遗传学模式的作用。在整个培养期间添加10⁻⁴ M DFO可使FA细胞的自发染色体断裂减少50%。此外,DFO也部分抵消了二环氧丁烷对FA淋巴细胞的致断裂作用。上述发现支持了这样一种假设,即FA发病机制中涉及的机制之一可能是此类患者的细胞清除活性氧的能力受损。讨论了FA患者白细胞内可螯合铁池与自由基形成之间的关系。

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