Loss of Baiap2l2 destabilizes the transducing stereocilia of cochlear hair cells and leads to deafness.

ABSTRACT:

Key points

Mechanoelectrical transduction at auditory hair cells requires highly specialized stereociliary bundles that project from their apical surface, forming a characteristic graded 'staircase' structure. The morphogenesis and maintenance of these stereociliary bundles is a tightly regulated process requiring the involvement of several actin-binding proteins, many of which are still unidentified. We identify a new stereociliary protein, the I-BAR protein BAIAP2L2, which localizes to the tips of the shorter transducing stereocilia in both inner and outer hair cells (IHCs and OHCs). We find that Baiap2l2 deficient mice lose their second and third rows of stereocilia, their mechanoelectrical transducer current, and develop progressive hearing loss, becoming deaf by 8 months of ag

SUBMITTER: Carlton AJ 

PROVIDER: S-EPMC7898316 | biostudies-literature | 2021 Feb

REPOSITORIES: biostudies-literature