Sox2 and inner ear development

作者：Junchang Li · 年份：2012 · DOI：10.5353/th_b4961745 · 研究领域：Hearing, Cochlea, Tinnitus, Genetics

Sox2, a HMG box transcription factor, is well known for its role in stem cell maintenance, iPS (induced pluripotent stem cell) induction, and development of neural tissues such as central nervous system and sensory organs. Sox2 has been demonstrated to be essential for the development of inner ear sensory patches. It has been shown that Sox2 is under the regulation of multiple regulatory elements to obtain a tissue specific manner.\n\nTwo allelic mouse mutants, yellow submarine (Ysb) and Light coat and circling (Lcc) show hearing and balance impairments with different severity. They were made by random insertions of a transgene (pAA2) and X-ray irradiation respectively. Ysb and Lcc are both localized to chromosome 3 and involve complex chromosomal rearrangements. The Sox2 coding region is intact in the mutants, while the Sox2 expression in the otocyst is greatly reduced in Ysb and totally lost in Lcc, which indicates the tissue specific reduction of Sox2 may be due to the rearrangement of Sox2 regulatory element(s). Since Sox2 null mutants die before implantation, the two Sox2 inner ear mutants are valuable models for studying Sox2 knock down (Ysb) and Sox2 knock out (Lcc) condition in the inner ear. To understand the molecular basis behind Sox2 regulation in the inner ear, this project aims to identify the Sox2 otic regulatory elements, and potential Sox2 downstream targets involved in the development of inner ear.\n\nPrevious work has indicated that Nop1 and Nop2 are the ot...