期刊
GENES
卷 12, 期 7, 页码 -出版社
MDPI
DOI: 10.3390/genes12071082
关键词
cochlea; inner ear; common marmoset; primate; connexin
资金
- Japanese government MEXT KAKENHI [18K16856, 20H03836, 21K19581, 18H04065, 19H05473]
- Keio Medical Association
- Society for Promotion of International Oto-Rhino-Laryngology (SPIO)
- Kanae Foundation for the Promotion of Medical Science
- Keio Gijuku Academic Development Funds
- Mitsubishi Tanabe
- Takeda Science Foundation
- Brain Mapping by Integrated Neurotechnologies for Disease Studies (Brain/MINDS)
- Keio University Medical Science Fund
- Grants-in-Aid for Scientific Research [21K19581, 20H03836, 18K16856, 18H04065] Funding Source: KAKEN
Connexins are crucial for normal hearing ability, with variants in the gene encoding connexin 26 being a common cause of congenital deafness. The study using common marmosets as a primate model reveals unique spatial and temporal expression patterns for connexins, suggesting primate-specific control and functions. Treatment approaches for connexin-related hearing loss in rodent models may not translate effectively to human patients, emphasizing the need for testing in primate models prior to clinical trials.
Connexins are gap junction components that are essential for acquiring normal hearing ability. Up to 50% of congenital, autosomal-recessive, non-syndromic deafness can be attributed to variants in GJB2, the gene that encodes connexin 26. Gene therapies modifying the expression of connexins are a feasible treatment option for some patients with genetic hearing losses. However, the expression patterns of these proteins in the human fetus are not fully understood due to ethical concerns. Recently, the common marmoset was used as a primate animal model for the human fetus. In this study, we examined the expression patterns of connexin 26 and connexin 30 in the developing cochlea of this primate. Primate-specific spatiotemporal expression changes were revealed, which suggest the existence of primate-specific control of connexin expression patterns and specific functions of these gap junction proteins. Moreover, our results indicate that treatments for connexin-related hearing loss established in rodent models may not be appropriate for human patients, underscoring the importance of testing these treatments in primate models before applying them in human clinical trials.
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