期刊
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
卷 10, 期 -, 页码 -出版社
FRONTIERS MEDIA SA
DOI: 10.3389/fcell.2022.950837
关键词
connexins; inner ear; inflammation; oxidative stress; vascular dysfunction; presbycusis; genetic risk factors
资金
- Consiglio Nazionale delle Ricerche (CNR, Italy) Progetto di Interesse Invecchiamento [DSB.AD009.001.004/INVECCHIAMENTO-IBCN]
- Fondazione Policlinico Universitario A. Gemelli
- INAIL Bando Bric 09. Universita Cattolica del Sacro Cuore
Deletion of Cx30 has been found to be associated with age-related hearing loss (ARHL). Mice with Cx30 deletion exhibit exacerbated ARHL, with increased cochlear damage, oxidative stress, inflammation, and vascular dysfunctions.
Pathogenic mutations in the Gjb2 and Gjb6 genes, encoding connexin 26 (Cx26) and connexin 30 (Cx30), respectively, have been linked to the most frequent monogenic hearing impairment, nonsyndromic hearing loss, and deafness DFNB1. It is known that Cx26 plays an important role in auditory development, while the role of Cx30 in hearing remains controversial. Previous studies found that partial deletion of Cx26 can accelerate age-related hearing loss (ARHL), a multifactorial complex disorder, with both environmental and genetic factors contributing to the etiology of the disease. Here, we investigated the role of Cx30 in cochlear-aging processes using a transgenic mouse model with total deletion of Cx30 (Cx30 delta delta mice), in which Cx30 was removed without perturbing the surrounding sequences. We show that these mice are affected by exacerbated ARHL, with increased morphological cochlear damage, oxidative stress, inflammation, and vascular dysfunctions. Overall, our data demonstrate that Cx30 deletion can be considered a genetic risk factor for ARHL, making cochlear structures more susceptible to aging processes.
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