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Abnormal Innervation, Demyelination, and Degeneration of Spiral Ganglion Neurons as Well as Disruption of Heminodes are Involved in the Onset of Deafness in Cx26 Null Mice.
- Source :
-
Neuroscience bulletin [Neurosci Bull] 2024 Aug; Vol. 40 (8), pp. 1093-1103. Date of Electronic Publication: 2024 Feb 04. - Publication Year :
- 2024
-
Abstract
- GJB2 gene mutations are the most common causes of autosomal recessive non-syndromic hereditary deafness. For individuals suffering from severe to profound GJB2-related deafness, cochlear implants have emerged as the sole remedy for auditory improvement. Some previous studies have highlighted the crucial role of preserving cochlear neural components in achieving favorable outcomes after cochlear implantation. Thus, we generated a conditional knockout mouse model (Cx26-CKO) in which Cx26 was completely deleted in the cochlear supporting cells driven by the Sox2 promoter. The Cx26-CKO mice showed severe hearing loss and massive loss of hair cells and Deiter's cells, which represented the extreme form of human deafness caused by GJB2 gene mutations. In addition, multiple pathological changes in the peripheral auditory nervous system were found, including abnormal innervation, demyelination, and degeneration of spiral ganglion neurons as well as disruption of heminodes in Cx26-CKO mice. These findings provide invaluable insights into the deafness mechanism and the treatment for severe deafness in Cx26-null mice.<br /> (© 2024. Center for Excellence in Brain Science and Intelligence Technology, Chinese Academy of Sciences.)
- Subjects :
- Animals
Demyelinating Diseases pathology
Demyelinating Diseases genetics
Mice
Neurons pathology
Neurons metabolism
Disease Models, Animal
Nerve Degeneration pathology
Nerve Degeneration genetics
Cochlea pathology
Spiral Ganglion pathology
Connexin 26
Deafness genetics
Deafness pathology
Mice, Knockout
Connexins genetics
Connexins deficiency
Subjects
Details
- Language :
- English
- ISSN :
- 1995-8218
- Volume :
- 40
- Issue :
- 8
- Database :
- MEDLINE
- Journal :
- Neuroscience bulletin
- Publication Type :
- Academic Journal
- Accession number :
- 38311706
- Full Text :
- https://doi.org/10.1007/s12264-023-01167-x