Dynamic Spatiotemporal Expression Changes in Connexins of the Developing Primate’s Cochlea

Genes

Makoto Hosoya¹  Masato Fujioka¹  Kaoru Ogawa¹  Hiroyuki Ozawa¹  Ayako Y. Murayama²  Hideyuki Okano² 

[1] Department of Otorhinolaryngology, Head and Neck Surgery, Keio University School of Medicine, 35 Shinanomachi Shinjuku-ku, Tokyo 160-8582, Japan;Department of Physiology, Keio University School of Medicine, 35 Shinanomachi Shinjuku-ku, Tokyo 160-8582, Japan;

关键词: cochlea;    inner ear;    common marmoset;    primate;    connexin;   

DOI  :  10.3390/genes12071082

来源: DOAJ

【 摘 要 】

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.

【 授权许可】

Unknown