Iida K, Koseki H, Kakinuma H, Kato N, Mizutani-Koseki Y, Ohuchi H, Yoshioka H, Noji S, Kawamura K, Kataoka Y, Ueno F, Taniguchi M, Yoshida N, Sugiyama T, Miura N
Department of Biochemistry, Akita University School of Medicine, Hondo, Japan.
Development. 1997 Nov;124(22):4627-38. doi: 10.1242/dev.124.22.4627.
Mesenchyme Fork Head-1 (MFH-1) is a forkhead (also called winged helix) transcription factor defined by a common 100-amino acid DNA-binding domain. MFH-1 is expressed in non-notochordal mesoderm in the prospective trunk region and in cephalic neural-crest and cephalic mesoderm-derived mesenchymal cells in the prechordal region of early embryos. Subsequently, strong expression is localized in developing cartilaginous tissues, kidney and dorsal aortas. To investigate the developmental roles of MFH-1 during embryogenesis, mice lacking the MFH-1 locus were generated by targeted mutagenesis. MFH-1-deficient mice died embryonically and perinatally, and exhibited interrupted aortic arch and skeletal defects in the neurocranium and the vertebral column. Interruption of the aortic arch seen in the mutant mice was the same as in human congenital anomalies. These results suggest that MFH-1 has indispensable roles during the extensive remodeling of the aortic arch in neural-crest-derived cells and in skeletogenesis in cells derived from the neural crest and the mesoderm.
间充质叉头蛋白-1（MFH-1）是一种叉头（也称为翼状螺旋）转录因子，由一个常见的100个氨基酸的DNA结合结构域定义。MFH-1在前体躯干区域的非脊索中胚层以及早期胚胎前索区域的头神经嵴和头中胚层来源的间充质细胞中表达。随后，强表达定位于发育中的软骨组织、肾脏和背主动脉。为了研究MFH-1在胚胎发生过程中的发育作用，通过靶向诱变产生了缺乏MFH-1基因座的小鼠。MFH-1缺陷小鼠在胚胎期和围产期死亡，并在神经颅骨和脊柱中表现出主动脉弓中断和骨骼缺陷。突变小鼠中出现的主动脉弓中断与人类先天性异常相同。这些结果表明，MFH-1在神经嵴来源细胞的主动脉弓广泛重塑以及神经嵴和中胚层来源细胞的骨骼发生过程中具有不可或缺的作用。
