Rbm24 regulates inner-ear-specific alternative splicing and is essential for maintaining auditory and motor coordination.

ABSTRACT: Tissue-specific alternative splicing (AS) is emerging as one of the most exciting types of mechanisms associated with organ development and disease. In the auditory system, many hearing-related genes undergo AS, and errors in this process result in syndromic or non-syndromic hearing loss. However, little is known about the factors and mechanisms directing AS in the inner ear. In the present study, we identified a novel RNA-binding protein, Rbm24, which was critically involved in regulating inner-ear-specific AS. Rbm24 deletion resulted in hearing loss and defects in motor coordination. Global splicing analysis showed Rbm24 was required for correct splicing of a subset of pre-mRNA transcripts with essential roles in stereocilia integrity and survival of hair cells. Furthermore, we identifie

SUBMITTER: Zheng L 

PROVIDER: S-EPMC7971244 | biostudies-literature | 2021 Apr

REPOSITORIES: biostudies-literature