Levanon Ditsa, Glusman Gustavo, Bettoun David, Ben-Asher Edna, Negreanu Varda, Bernstein Yael, Harris-Cerruti Catherine, Brenner Ori, Eilam Raya, Lotem Joseph, Fainaru Ofer, Goldenberg Dalia, Pozner Amir, Woolf Eilon, Xiao Cuiying, Yarmus Merav, Groner Yoram
Department of Molecular Genetics, Weizmann Institute of Science, Rehovot 76100, Israel.
Blood Cells Mol Dis. 2003 Mar-Apr;30(2):161-3. doi: 10.1016/s1079-9796(03)00023-8.
The RUNX transcription factors are key regulators of lineage specific gene expression in developmental pathways. The mammalian RUNX genes arose early in evolution and maintained extensive structural similarities. Sequence analysis suggested that RUNX3 is the most ancient of the three mammalian genes, consistent with its role in neurogenesis of the monosynaptic reflex arc, the simplest neuronal response circuit, found in Cnidarians, the most primitive animals. All RUNX proteins bind to the same DNA motif and act as activators or repressors of transcription through recruitment of common transcriptional modulators. Nevertheless, analysis of Runx1 and Runx3 expression during embryogenesis revealed that their function is not redundant. In adults both Runx1 and Runx3 are highly expressed in the hematopoietic system. At early embryonic stages we found strong Runx3 expression in dorsal root ganglia neurons, confined to TrkC sensory neurons. In the absence of Runx3, knockout mice develop severe ataxia due to the early death of the TrkC neurons. Other phenotypic defects of Runx3 KO mice including abnormalities in thymopoiesis are also being investigated.
RUNX转录因子是发育途径中谱系特异性基因表达的关键调节因子。哺乳动物的RUNX基因在进化早期出现,并保持了广泛的结构相似性。序列分析表明,RUNX3是三个哺乳动物基因中最古老的,这与其在单突触反射弧神经发生中的作用一致,单突触反射弧是在最原始的动物刺胞动物中发现的最简单的神经元反应回路。所有RUNX蛋白都与相同的DNA基序结合,并通过募集共同的转录调节因子作为转录激活剂或抑制剂发挥作用。然而,对胚胎发育过程中Runx1和Runx3表达的分析表明它们的功能并非冗余。在成年个体中,Runx1和Runx3在造血系统中均高度表达。在胚胎早期阶段,我们发现Runx3在背根神经节神经元中强烈表达,仅限于TrkC感觉神经元。在缺乏Runx3的情况下,基因敲除小鼠由于TrkC神经元的早期死亡而出现严重共济失调。Runx3基因敲除小鼠的其他表型缺陷,包括胸腺生成异常,也正在研究中。