Postnatal Refinement of Auditory Hair Cell Planar Polarity Deficits Occurs in the Absence of Vangl2

• DOI: 10.1523/jneurosci.1307-13.2013
• Publication Date: 2013-08-28T16:33:41Z
• Authors: C. O. Copley; J. S. Duncan; C. Liu; H. Cheng; M. R. Deans
• Keywords: Polarity (international relations); Cell polarity

The distinctive planar polarity of auditory hair cells is evident in the polarized organization stereociliary bundle. Mutations core cell gene Van Gogh-like 2 (<i>Vangl2</i>) result that fail to properly orient their bundles along mediolateral axis cochlea. severity this phenotype graded length cochlea, similar differentiation gradient, suggesting an active refinement process corrects phenotypes <i>Vangl2</i> knock-out (KO) mice. Because deletions are lethal, conditional knock-outs (CKOs) were generated test hypothesis. When crossed with <i>Pax2–Cre</i>, deleted from inner ear, yielding KOs at late embryonic stages except CKO mice viable and do not have craniorachischisis like KOs. Quantification deficits through postnatal development demonstrates activity a <i>Vangl2-</i>independent rescues within 10 d birth. In contrast, <i>Pax2–Cre</i>;<i>Vangl2</i> has profound changes shape distribution outer pillar Deiters9 phalangeal processes corrected during period refinement. Auditory brainstem response analyses adult show 10–15 dB shift threshold, distortion product otoacoustic emission measurements indicate mild hearing deficit cochlear origin. Together, these data demonstrate <i>Vangl2</i>-independent mechanism actively reorients reveals unexpected role supporting morphogenesis.