ORIGINAL RESEARCH article
Front. Mol. Neurosci.
Sec. Brain Disease Mechanisms
Volume 18 - 2025 | doi: 10.3389/fnmol.2025.1604262
This article is part of the Research TopicMolecular Neuroscience: Current and Future ChallengesView all articles
Delayed structural maturation of inner hair cell ribbon synapses in a mouse model of fragile X syndrome
Provisionally accepted
- University of Warsaw, Warsaw, Poland
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Clinical features of the FXS phenotype include intellectual disability, repetitive behaviors, social communication deficits, and, commonly, auditory hypersensitivity to acoustic stimuli.Electrophysiological studies have shown that FXS patients and Fmr1KO mice exhibit improper processing of auditory information in the cortical areas of the brain and the spiral ganglion of the 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 structural maturation in Fmr1 KO mice. Interestingly, the ultrastructure of inner hair cell ribbon synapses, studied by electron microscopy in adult mice (P48), has shown no specific dysmorphologies. Delayed structural maturation of presynaptic ribbons of auditory hair cells in Fmr1 KO mice may contribute to abnormal development of circuits induced by auditory experience.
Keywords: Fragile X syndrome -FMRP, inner hair cell, ribbon synapses, Electron microscopy, Organ of Corti
Received: 01 Apr 2025; Accepted: 01 Aug 2025.
Copyright: © 2025 Chojnacka, Skupien-Jaroszek and Dziembowska. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Magdalena Dziembowska, University of Warsaw, Warsaw, Poland
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