Loss of liver kinase B1 causes planar polarity defects in cochlear hair cells in mice

作者：Men, Yuqin; Zhang, Aizhen; Zhang, Liwen; Jin, Yecheng; Wang, Zhishuo; Zhao, Jing; Yu, Xiaolin; Zhang, Jian^*; Gao, Jiangang^*

来源：Frontiers of Medicine, 2016, 10(4): 481-489.

DOI：10.1007/s11684-016-0494-3

• LKB1
• stereociliary bundles
• kinocilium
• planar cell polarity
• hearing
• mice

The tumor suppressor gene liver kinase B1 (LKB1), also called STK11, encodes a serine/threonine kinase. LKB1 plays crucial roles in cell differentiation, proliferation, and polarity. In this study, LKB1 conditional knockout mice (LKB1(Pax2) CKO mice) were generated using Pax2-Cre mice to investigate the function of LKB1 in inner ear hair cells during early embryonic period. LKB1(Pax2) CKO mice died perinatally. Immunofluorescence and scanning electron microscopy revealed that stereociliary bundles in LKB1(Pax2) CKO mice were clustered and misoriented, respectively. Moreover, ectopic distribution of kinocilium bundles resulting from abnormal migration of kinocilium was observed in the mutant mice. The orientation of stereociliary bundles and the migration of kinocilia are critical indicators of planar cell polarity (PCP) of hair cells. LKB1 deficiency in LKB1(Pax2) CKO mice thus disrupted hair cell planar polarity during embryonic development. Our results suggest that LKB1 is required in PCP formation in cochlear hair cells in mice.

• 出版日期2016-12
• 单位山东大学