Deterioration of the Medial Olivocochlear Efferent System Accelerates Age-Related Hearing Loss in Pax2-Isl1 Transgenic Mice

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• 作者：Tetyana Chumak ; Romana Bohuslavova ; Iva Macova ; Nicole Dodd…
• 关键词：Medial olivocochlear efferent system ; Islet1 transcription factor ; Age ; related hearing loss ; Outer hair cells ; Transgenic mouse
• 刊名：Molecular Neurobiology
• 出版年：2016
• 出版时间：May 2016
• 年：2016
• 卷：53
• 期：4
• 页码：2368-2383
• 全文大小：6,610 KB
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• 作者单位：Tetyana Chumak (1)
  Romana Bohuslavova (2)
  Iva Macova (2)
  Nicole Dodd (2)
  Daniela Buckiova (1)
  Bernd Fritzsch (3)
  Josef Syka (1)
  Gabriela Pavlinkova (2)
  
  1. Institute of Experimental Medicine, CAS, Prague, Czechia
  2. Laboratory of Molecular Pathogenetics, Institute of Biotechnology, CAS, Prague 4, CZ-142 20, Prague, Czechia
  3. Department of Biology, University of Iowa, Iowa City, IA, 52242, USA
  
• 刊物主题：Neurosciences; Neurobiology; Cell Biology; Neurology;
• 出版者：Springer US
• ISSN：1559-1182

文摘

The development, maturation, and maintenance of the inner ear are governed by temporal and spatial expression cascades of transcription factors that form a gene regulatory network. ISLET1 (ISL1) may be one of the major players in this cascade, and in order to study its role in the regulation of inner ear development, we produced a transgenic mouse overexpressing Isl1 under the Pax2 promoter. Pax2-regulated ISL1 overexpression increases the embryonic ISL1+ domain and induces accelerated nerve fiber extension and branching in E12.5 embryos. Despite these gains in early development, the overexpression of ISL1 impairs the maintenance and function of hair cells of the organ of Corti. Mutant mice exhibit hyperactivity, circling behavior, and progressive age-related decline in hearing functions, which is reflected in reduced otoacoustic emissions (DPOAEs) followed by elevated hearing thresholds. The reduction of the amplitude of DPOAEs in transgenic mice was first detected at 1 month of age. By 6–9 months of age, DPOAEs completely disappeared, suggesting a functional inefficiency of outer hair cells (OHCs). The timing of DPOAE reduction coincides with the onset of the deterioration of cochlear efferent terminals. In contrast to these effects on efferents, we only found a moderate loss of OHCs and spiral ganglion neurons. For the first time, our results show that the genetic alteration of the medial olivocochlear (MOC) efferent system induces an early onset of age-related hearing loss. Thus, the neurodegeneration of the MOC system could be a contributing factor to the pathology of age-related hearing loss.