Functions of the runt gene family in development

PEBP 2转录因子作为由α和β亚基组成的异二聚体发挥功能。α亚基负责PEBP 2的DNA结合活性，其通过与β亚基相互作用而进一步增强。AML 1基因编码PEBP 2的α亚基，与果蝇runt基因的哺乳动物同源物相对应，该基因参与果蝇的分节、神经发育和性别决定。编码PEBP 2 β亚基的哺乳动物PEBP 2 β基因代表一个矮小配偶体基因。AMI.I和PEBP 2 β基因均参与与人类急性髓性白血病相关的染色体重排。本研究旨在揭示哺乳动物runt和runt-partner基因在细胞分化、转化和发育中的功能。结果表明：1）PEBP 2转录因子在胚胎造血发育过程中是不可缺少的，并以剂量依赖的方式发挥作用; 2）与人AML相关的嵌合基因的产物，包括PEBP 2 β基因，损害了细胞质的细胞结构; 3）AML 1转录因子的剂量对决定T细胞受体的质量至关重要。介导的信号对细胞存活或细胞死亡的影响。

The PEBP2 transcription factor functions as a heterodimer composed of the alpha and beta subunits. The alpha subunit is responsible for the DNA binding activity of PEBP2, which is furthermore enhanced by interacting with the beta subunit. The AML1 gene, encoding the alpha subunit of PEBP2, corresponds to the mammalian homologue of Drosophila runt gene, a gene involved in Segmentation, neural development and sex determination in fly. The mammalian PEBP2beta gene encoding the beta subunit of PEBP2 represents a runt-partner gene. Both the AMI.I and PEBP2beta genes are involved in chromosomal rearrangements associated which human acute myeloid leukemia. As a result of translocation and/or inversion, the chimeric genes are generated.The study was carried out to reveal the functions of mammalian runt and runt-partner genes in cell differentiation, transformation and development. The results are summarized as follows.l)The PEBP2 transcription factor is indispensable and functions in a dose dependent manner for the hematopoietic development in the embryonic aorta-gonad-mesonephros region.2)The product of the chimeric gene associate with human AML and involving the PEBP2beta gene impairs the architecture of cytoplasmic cytoskeleton.3)A dose of AML1 transcription factor is critical in deternuning the quality of T cell receptor-mediated signal to cell survival or cell death.