Chen You-Tzung, Akinwunmi Peter O, Deng Jian Min, Tam Oliver H, Behringer Richard R
Department of Molecular Genetics, University of Texas M. D. Anderson Cancer Center, Houston, Texas 77030, USA.
Genesis. 2007 Sep;45(9):588-92. doi: 10.1002/dvg.20332.
Twist1 is the mouse ortholog of TWIST1, the human gene mutated in Saethre-Chotzen syndrome. Previously, a Twist1 null allele was generated by gene targeting in mouse embryonic stem cells. Twist1 heterozygous mice develop polydactyly and a craniofacial phenotype similar to Saethre-Chotzen patients. Mice homozygous for the Twist1 null allele die around embryonic day 11.5 (E11.5) with cranial neural tube closure and vascular defects, hindering in vivo studies of Twist1 function at later stages of development. Here, we report the generation of a Twist1 conditional null allele in mice that functions like a wild-type allele but can be converted to a null allele upon Cre-mediated recombination.
Twist1是人类基因TWIST1在小鼠中的直系同源基因,该人类基因在塞特勒-乔岑综合征中发生突变。此前,通过在小鼠胚胎干细胞中进行基因打靶产生了一个Twist1无效等位基因。Twist1杂合小鼠会出现多指畸形以及与塞特勒-乔岑综合征患者相似的颅面表型。Twist1无效等位基因的纯合小鼠在胚胎第11.5天(E11.5)左右死亡,伴有颅神经管闭合和血管缺陷,这阻碍了对Twist1在发育后期功能的体内研究。在此,我们报告了在小鼠中产生的一个Twist1条件性无效等位基因,其功能类似于野生型等位基因,但在Cre介导的重组作用下可转变为无效等位基因。