Characterization of the Expression and Role of Striatin-Interacting Protein 2 in Mouse Cochlea.

IF 1.9 3区 医学 Q3 CLINICAL NEUROLOGY
Otology & Neurotology Pub Date : 2025-04-01 Epub Date: 2025-02-14 DOI:10.1097/MAO.0000000000004449
Siyu Li, Yue Qiu, Ao Li, Jie Lu, Xinya Ji, Wenli Hao, Cheng Cheng, Xia Gao
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Abstract

Hypothesis: In this study, we aimed to examine the cochlear expression pattern and function of Striatin-interacting protein 2 (STRIP2) by using animal models.

Background: Sensorineural hearing loss often results from genetic defects in hair cell (HC) development and function. STRIP2 is a part of the striatin-interacting phosphatase and kinase (STRIPAK) complex, which plays important regulatory roles in cell fate determination, proliferation, cytoskeletal organization, and cell morphology. A recent study revealed Strip2 as the candidate gene that regulates positive selection in HC lineages. However, its role in the inner ear has not been identified.

Methods: Strip2 knockout mouse model was used to examine the cochlear expression pattern and function of STRIP2. Auditory brainstem response test was used to evaluate the hearing function of mice. Immunostaining and scanning electron microscope were used to study hair cells, synapses, and stereocilia of cochlea.

Results: Immunostaining showed that cytoplasmic STRIP2 expression in hair cells increased from postnatal day (P) 3 to P14. Despite having normal hearing thresholds, hair cell numbers, and stereocilia morphology until P90, the deletion of Strip2 resulted in a mild reduction in ribbon synapse density, suggesting a late onset of cochlear synaptic defects.

Conclusion: Our results revealed that STRIP2 was abundantly expressed in hair cells; however, the hearing function of Strip2-/- mice was comparable to that of control mice until P90, and a mild decrease in ribbon synapse number was detected at P60 and P90. Further studies on STRIP2 and its associated complexes will provide new insights into the pathways involved in inner ear development and function.

纹状蛋白相互作用蛋白2在小鼠耳蜗中的表达及作用。
假设:在本研究中,我们旨在通过动物模型研究耳蜗纹状蛋白相互作用蛋白2 (Striatin-interacting protein 2, STRIP2)的表达模式和功能。背景:感音神经性听力损失通常是由毛细胞发育和功能的遗传缺陷引起的。STRIP2是纹状蛋白相互作用磷酸酶和激酶(STRIPAK)复合物的一部分,在细胞命运决定、增殖、细胞骨架组织和细胞形态中起重要的调节作用。最近的一项研究表明,Strip2是HC谱系中调节阳性选择的候选基因。然而,它在内耳中的作用尚未确定。方法:采用Strip2基因敲除小鼠模型,检测耳蜗中Strip2基因的表达模式和功能。采用听觉脑干反应试验评价小鼠的听力功能。采用免疫染色和扫描电镜对耳蜗毛细胞、突触和立体纤毛进行了研究。结果:免疫染色显示,毛细胞胞浆中STRIP2的表达从出生后第3天到第14天增加。尽管在P90之前具有正常的听力阈值、毛细胞数量和纤毛形态,但Strip2的缺失导致带状突触密度轻度降低,提示耳蜗突触缺陷的晚发性。结论:我们的研究结果显示,STRIP2在毛细胞中大量表达;然而,直到P90, Strip2-/-小鼠的听力功能与对照小鼠相当,并且在P60和P90时检测到带状突触数量轻度减少。对STRIP2及其相关复合物的进一步研究将为内耳发育和功能的通路提供新的见解。
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来源期刊
Otology & Neurotology
Otology & Neurotology 医学-耳鼻喉科学
CiteScore
3.80
自引率
14.30%
发文量
509
审稿时长
3-6 weeks
期刊介绍: ​​​​​Otology & Neurotology publishes original articles relating to both clinical and basic science aspects of otology, neurotology, and cranial base surgery. As the foremost journal in its field, it has become the favored place for publishing the best of new science relating to the human ear and its diseases. The broadly international character of its contributing authors, editorial board, and readership provides the Journal its decidedly global perspective.
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