Ribosomal protein S19 deficiency leads to reduced proliferation and increased apoptosis but does not affect terminal erythroid differentiation in a cell line model of Diamond-Blackfan anemia
Ribosomal protein S19 deficiency leads to reduced proliferation and increased apoptosis but does not affect terminal erythroid differentiation in a cell line model of Diamond-Blackfan anemia
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DOI：
10.1634/stemcells.2007-0569
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发表时间：
2008-02-01
期刊：
影响因子：
5.2
通讯作者：
Karlsson, Stefan
中科院分区：
文献类型：
--
作者：
Miyake, Koich;Utsugisawa, Taiju;Karlsson, Stefan
Diamond-Blackfan贫血（DBA）是一种先天性红细胞发育不全，其中25%的患者存在核糖体蛋白（RP）S19基因突变。目前尚不清楚RPS 19缺陷如何损害红细胞生成和造血祖细胞的增殖。为了阐明RPS 19缺陷DBA的分子机制，我们分析了RPS 19缺陷对促红细胞生成素（EPO）诱导的信号转导、细胞周期和RPS 19缺陷TF-1细胞凋亡的影响。未发现EPO诱导的信号转导异常。然而，RPS 19缺陷型TF-1细胞表现出G 0/G1期阻滞（82%对58%; p
Diamond-Blackfan anemia (DBA) is a congenital red-cell aplasia in which 25% of the patients have a mutation in the ribosomal protein (RP) S19 gene. It is not known how the RPS19 deficiency impairs erythropoiesis and proliferation of hematopoietic progenitors. To elucidate molecular mechanisms in RPS19-deficient DBA, we analyzed the effects of RPS19 deficiency on erythropoietin (EPO)-induced signal transduction, cell cycle, and apoptosis in RPS19-deficient TF-1 cells. We did not rind any abnormality in EPO-induced signal transduction. However, RPS19-deficient TF-1 cells showed G0/G1 arrest (82% vs. 58%; p