Hearing impairment due to <i>Mir183/96/182</i> mutations suggests both loss and gain of function effects.

ABSTRACT: The microRNA miR-96 is important for hearing, as point mutations in humans and mice result in dominant progressive hearing loss. Mir96 is expressed in sensory cells along with Mir182 and Mir183, but the roles of these closely-linked microRNAs are as yet unknown. Here we analyse mice carrying null alleles of Mir182, and of Mir183 and Mir96 together to investigate their roles in hearing. We found that Mir183/96 heterozygous mice had normal hearing and homozygotes were completely deaf with abnormal hair cell stereocilia bundles and reduced numbers of inner hair cell synapses at four weeks old. Mir182 knockout mice developed normal hearing then exhibited progressive hearing loss. Our transcriptional analyses revealed significant ch

SUBMITTER: Lewis MA 

PROVIDER: S-EPMC7903918 | biostudies-literature | 2020 Dec

REPOSITORIES: biostudies-literature