Pitx2 regulates lung asymmetry, cardiac positioning and pituitary and tooth morphogenesis
Pitx2 regulates lung asymmetry, cardiac positioning and pituitary and tooth morphogenesis
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DOI：
10.1038/45803
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发表时间：
1999-09-16
期刊：
影响因子：
64.8
通讯作者：
Rosenfeld, MG
中科院分区：
文献类型：
--
作者：
Lin, CR;Kioussi, C;Rosenfeld, MG
Pitx1（参考文献 1-3）和 Pitx2（参考文献 4、5）是高度同源的、与 bicoid 相关的转录因子。 Pitx2 最初被确定为导致人类 Rieger 综合征 (4) 的基因，这是一种导致发育异常的常染色体显性病症。 Pitx2 在左侧板中胚层 (5-11) 中不对称表达，具有偏侧性缺陷的突变小鼠表现出与内脏对称性（位置）变化相关的 Pitx2 表达模式的改变。 Pitx2 在右侧板中胚层中的异位表达会改变小鸡和非洲爪蟾胚胎中心脏和肠道的循环并逆转身体旋转(6-11)。在这里，我们描述了 Pitx2 基因缺失小鼠的表型，其特征是体壁闭合缺陷、右肺异构、心脏位置改变、转动停滞，以及随后垂体前叶和牙齿器官发生的确定和增殖事件受阻。因此，Pitx2 是一种编码肺“左性”的转录因子。
Pitx1 (refs 1-3) and Pitx2 (refs 4, 5) are highly homologous, bicoid-related transcription factors. Pitx2 was initially identified as the gene responsible for the human Rieger syndrome(4), an autosomal dominant condition that causes developmental abnormalities. Pitx2 is asymmetrically expressed in the left lateral-plate mesoderm(5-11), and mutant mice with laterality defects show altered patterns of Pitx2 expression that correlate with changes in the visceral symmetry (situs). Ectopic expression of Pitx2 in the right lateral-plate mesoderm alters looping of the heart and gut and reverses body rotation in chick and Xenopus embryose(6-11). Here we describe the phenotype of Pitx2 gene-deleted mice, characterized by defective body-wall closure, right pulmonary isomerism, altered cardiac position, arrest in turning and, subsequently a block in the determination and proliferation events of anterior pituitary gland and tooth organogenesis. Thus, Pitx2 is a transcription factor that encodes 'leftness' of the lung.