Department of Pathology, Stanford University School of Medicine, Stanford, CA 94305, USA.
Blood. 2012 Jul 5;120(1):76-85. doi: 10.1182/blood-2011-12-399113. Epub 2012 May 18.
Runx1(P1N/P1N) mice are deficient in the transcription factor distal promoter-derived Runt-related transcription factor 1 (P1-Runx1) and have a > 90% reduction in the numbers of basophils in the BM, spleen, and blood. In contrast, Runx1(P1N/P1N) mice have normal numbers of the other granulocytes (neutrophils and eosinophils). Although basophils and mast cells share some common features, Runx1(P1N/P1N) mice have normal numbers of mast cells in multiple tissues. Runx1(P1N/P1N) mice fail to develop a basophil-dependent reaction, IgE-mediated chronic allergic inflammation of the skin, but respond normally when tested for IgE- and mast cell-dependent passive cutaneous anaphylaxis in vivo or IgE-dependent mast cell degranulation in vitro. These results demonstrate that Runx1(P1N/P1N) mice exhibit markedly impaired function of basophils, but not mast cells. Infection with the parasite Strongyloides venezuelensis and injections of IL-3, each of which induces marked basophilia in wild-type mice, also induce modest expansions of the very small populations of basophils in Runx1(P1N/P1N) mice. Finally, Runx1(P1N/P1N) mice have normal numbers of the granulocyte progenitor cells, SN-Flk2(+/-), which can give rise to all granulocytes, but exhibit a > 95% reduction in basophil progenitors. The results of the present study suggest that P1-Runx1 is critical for a stage of basophil development between SN-Flk2(+/-) cells and basophil progenitors.
Runx1(P1N/P1N) 小鼠缺乏转录因子远端启动子衍生的 Runt 相关转录因子 1 (P1-Runx1),骨髓、脾脏和血液中的嗜碱性粒细胞数量减少超过 90%。相比之下,Runx1(P1N/P1N) 小鼠的其他粒细胞(中性粒细胞和嗜酸性粒细胞)数量正常。尽管嗜碱性粒细胞和肥大细胞具有一些共同特征,但 Runx1(P1N/P1N) 小鼠在多种组织中的肥大细胞数量正常。Runx1(P1N/P1N) 小鼠无法发展出依赖嗜碱性粒细胞的反应、IgE 介导的皮肤慢性过敏炎症,但在体内检测 IgE 和肥大细胞依赖性被动皮肤过敏反应或体外 IgE 依赖性肥大细胞脱颗粒时,它们的反应正常。这些结果表明,Runx1(P1N/P1N) 小鼠表现出明显的嗜碱性粒细胞功能受损,但肥大细胞功能正常。寄生虫 Strongyloides venezuelensis 的感染和白细胞介素 3 的注射,这两种情况都会在野生型小鼠中引起明显的嗜碱性粒细胞增多,也会导致 Runx1(P1N/P1N) 小鼠中非常少量的嗜碱性粒细胞适度扩张。最后,Runx1(P1N/P1N) 小鼠的粒细胞祖细胞 SN-Flk2(+/-)数量正常,这些细胞可以产生所有的粒细胞,但嗜碱性粒细胞祖细胞减少超过 95%。本研究结果表明,P1-Runx1 对于 SN-Flk2(+/-)细胞和嗜碱性粒细胞祖细胞之间的嗜碱性粒细胞发育阶段至关重要。