Department of Genetics and Genomic Sciences, Mount Sinai School of Medicine, New York, NY 10029, USA.
Hear Res. 2013 Mar;297:13-9. doi: 10.1016/j.heares.2012.09.009. Epub 2012 Oct 24.
The phosphatase-transactivator EYA1 interacts with the homeodomain protein SIX1 to form transcriptional activation complexes, which play essential roles in regulating cell proliferation, survival and induction of sensory and neuronal differentiation programs during inner ear development. Mutations of the Eya1 and Six1 genes cause profound developmental auditory defects in mice and humans. The molecular mechanisms and developmental processes controlled by the EYA1 and SIX1 complex in inner ear development and neurosensory fate induction are the focus of this review.
磷酸酶激活因子 EYA1 与同源盒蛋白 SIX1 相互作用形成转录激活复合物,在调控内耳发育过程中的细胞增殖、存活以及感觉神经元和神经分化程序中发挥着重要作用。Eya1 和 Six1 基因突变会导致小鼠和人类严重的听觉发育缺陷。本文主要综述 EYA1 和 SIX1 复合物在内耳发育和感觉神经元命运诱导中所调控的分子机制和发育过程。