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April 1st, 2025
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University of Warsaw, Faculty of Biology, Institute of Experimental Zoology, Warsaw, Poland
neuroscience
biorxiv

Delayed development of inner hair cell ribbon synapses in a mouse model of fragile X syndrome

Chojnacka, M.Open in Google Scholar•Skupien-Jaroszek, A.Open in Google Scholar•Dziembowska, M.Open in Google Scholar

Clinical features of FXS phenotype include intellectual disability, repetitive behaviors, social communication deficit and also commonly manifested auditory hypersensitivity for acoustic stimuli. The electrophysiological studies have shown that FXS patients and Fmr1KO mice display improper processing of auditory information in the cortical areas of the brain and the spiral ganglion of cochlea. Synapses formed by spiral ganglion neurons on sensory hair cells (HC) are the first connection on the path that conveys the auditory information from the sensory cells to the brain. We confirmed the presence of fragile X mental retardation protein (FMRP) in the inner hair cells of the cochlea. Next, we analyzed the morphology of IHC ribbon synapses in early stages of postnatal development (P5, P14) and detected their delayed development in Fmr1 KO mice. Interestingly the ultrastructure of inner hair cell ribbon synapses studied by electron microscopy in the adult mice have shown no specific dysmorphologies. Delayed development of presynaptic ribbons of auditory hair cells in Fmr1 KO mice may contribute to abnormal development of circuits induced by auditory experience.

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