Rb 与造血：从干细胞到贫血。
Rb and hematopoiesis: stem cells to anemia.
机构信息
Department of Pediatric Oncology, Dana-Farber Cancer Institute, Division of Hematology/Oncology and Stem Cell Program, Children's Hospital Boston, Harvard Stem Cell Institute, Harvard Medical School, Boston, MA, 02115, USA.
St. Vincent's Institute, Department of Medicine at St. Vincent's Hospital, University of Melbourne, Fitzroy, VIC 3065, Australia.
出版信息
Cell Div. 2008 Sep 8;3:13. doi: 10.1186/1747-1028-3-13.
The retinoblastoma protein, Rb, was one of the first tumor suppressor genes identified as a result of the familial syndrome retinoblastoma. In the period since its identification and cloning a large number of studies have described its role in various cellular processes. The application of conditional somatic mutation with lineage and temporally controlled gene deletion strategies, thus circumventing the lethality associated with germ-line deletion of Rb, have allowed for a reanalysis of the in vivo role of Rb. In the hematopoietic system, such approaches have led to new insights into stem cell biology and the role of the microenvironment in regulating hematopoietic stem cell fate. They have also clarified the role that Rb plays during erythropoiesis and defined a novel mechanism linking mitochondrial function to terminal cell cycle withdrawal. These studies have shed light on the in vivo role of Rb in the regulation of hematopoiesis and also prompt further analysis of the role that Rb plays in both the regulation of hematopoietic stem cells and the terminal differentiation of their progeny.
视网膜母细胞瘤蛋白（Rb）是因家族性视网膜母细胞瘤综合征而被首次鉴定为肿瘤抑制基因的蛋白之一。自其被鉴定和克隆以来，大量研究已经描述了其在各种细胞过程中的作用。通过条件性体细胞突变与谱系和时间控制的基因缺失策略的应用，从而规避与 Rb 种系缺失相关的致死性，使得对 Rb 体内作用的重新分析成为可能。在造血系统中，这些方法为干细胞生物学和微环境在调节造血干细胞命运中的作用提供了新的见解。它们还阐明了 Rb 在红细胞生成过程中的作用，并定义了一个将线粒体功能与终末细胞周期退出联系起来的新机制。这些研究揭示了 Rb 在调节造血中的体内作用，并促使进一步分析 Rb 在调节造血干细胞及其后代的终末分化中的作用。
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