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<p>The mammalian cochlea relies on outer and inner hair cells (OHCs/IHCs) for sound amplification and signal transmission. Rab3-interacting molecular binding protein 2 (RIMBP2), expressed in receptor cells and neurons at synaptic active zones, remains poorly characterized in hearing. We therefore generated a <i>Rimbp2</i> knockout (KO) mouse model (<i>Rimbp2</i><sup><i>-/-</i></sup>), which exhibited severe hearing loss with elevated thresholds, prolonged latencies, and reduced amplitudes in auditory brainstem response Wave I. OHC loss <i>via</i> apoptosis was correlated with threshold elevation. In IHCs, patch-clamp recordings revealed reduced exocytosis, including a diminished readily-releasable pool, impaired sustained release, and blocked fast endocytosis. Immunostaining showed unchanged ribbon synapse numbers but positional shifts in the basal pole of KO IHCs. These findings demonstrated RIMBP2’s essential role in OHC survival and its broader regulatory functions in IHC synaptic transmission than previously recognized.</p>

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Multifaceted Role of RIMBP2 in Promoting Hearing in Murine Cochlear Hair Cells

  • 期刊论文

  • 发表日期：

    2025年8月29日

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  • Menghui Liao,
  • Xin Chen,
  • Ling Lu,
  • Qing Liu,
  • Rongrong Guo,
  • Yuyang Qiu,
  • Yangnan Hu,
  • Yuhua Zhang,
  • Qiaojun Fang,
  • Panpan Zhang,
  • Yige Li,
  • Shuijin He,
  • Mingliang Tang,
  • Huawei Li,
  • Geng-Lin Li,
  • Renjie Chai

摘要

The mammalian cochlea relies on outer and inner hair cells (OHCs/IHCs) for sound amplification and signal transmission. Rab3-interacting molecular binding protein 2 (RIMBP2), expressed in receptor cells and neurons at synaptic active zones, remains poorly characterized in hearing. We therefore generated a Rimbp2 knockout (KO) mouse model (Rimbp2-/-), which exhibited severe hearing loss with elevated thresholds, prolonged latencies, and reduced amplitudes in auditory brainstem response Wave I. OHC loss via apoptosis was correlated with threshold elevation. In IHCs, patch-clamp recordings revealed reduced exocytosis, including a diminished readily-releasable pool, impaired sustained release, and blocked fast endocytosis. Immunostaining showed unchanged ribbon synapse numbers but positional shifts in the basal pole of KO IHCs. These findings demonstrated RIMBP2’s essential role in OHC survival and its broader regulatory functions in IHC synaptic transmission than previously recognized.