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Evaluation of Planar-Cell-Polarity Phenotypes in Ciliopathy Mouse Mutant Cochlea
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Evaluation of Planar-Cell-Polarity Phenotypes in Ciliopathy Mouse Mutant Cochlea

Published on: February 21, 2016

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

Yuqin Men1, Aizhen Zhang1, Liwen Zhang2

  • 1School of Life Science and Key Laboratory of the Ministry of Education for Experimental Teratology, Shandong University, Jinan, 250100, China.

Frontiers of Medicine
|November 30, 2016
PubMed

Insights

Liver kinase B1 (LKB1) is essential for inner ear hair cell development. LKB1 deficiency disrupts planar cell polarity, leading to abnormal stereocilia and kinocilia, crucial for hearing.

Area of Science:

  • Developmental Biology
  • Otolaryngology
  • Genetics

Background:

  • Liver kinase B1 (LKB1), a serine/threonine kinase, is a crucial tumor suppressor gene.
  • LKB1 regulates fundamental cellular processes including differentiation, proliferation, and polarity.
  • Inner ear hair cell development relies on precise cellular organization for auditory function.

Purpose of the Study:

  • To investigate the role of LKB1 in the development of inner ear hair cells.
  • To examine the impact of LKB1 deficiency on hair cell planar cell polarity (PCP) during embryogenesis.

Main Methods:

  • Generation of LKB1 conditional knockout mice (LKB1Pax2 CKO) using Pax2-Cre driver lines.
  • Analysis of inner ear hair cell morphology using immunofluorescence and scanning electron microscopy.
  • Evaluation of stereociliary bundle orientation and kinocilium migration in mutant mice.

Main Results:

  • LKB1Pax2 CKO mice exhibited perinatal lethality.
  • Stereociliary bundles were clustered and misoriented; kinocilium bundles showed ectopic distribution due to abnormal migration.
  • LKB1 deficiency led to disrupted planar cell polarity in cochlear hair cells.

Conclusions:

  • LKB1 is indispensable for establishing planar cell polarity in mouse cochlear hair cells.
  • Disruption of LKB1 function severely impacts hair cell structure and organization during embryonic development.
  • These findings highlight LKB1's critical role in the development of the auditory system.
Keywords:
LKB1hearingkinociliummiceplanar cell polaritystereociliary bundles

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