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

被引:11
作者
Hosoya, Makoto [1 ]
Fujioka, Masato [1 ]
Murayama, Ayako Y. [2 ,3 ]
Ogawa, Kaoru [1 ]
Okano, Hideyuki [2 ,3 ]
Ozawa, Hiroyuki [1 ]
机构
[1] Keio Univ, Sch Med, Dept Otorhinolaryngol Head & Neck Surg, Shinjuku Ku, 35 Shinanomachi, Tokyo 1608582, Japan
[2] Keio Univ, Sch Med, Dept Physiol, Shinjuku Ku, 35 Shinanomachi, Tokyo 1608582, Japan
[3] RIKEN Ctr Brain Sci, Lab Marmoset Neural Architecture, Wako, Saitama 3510198, Japan
关键词
cochlea; inner ear; common marmoset; primate; connexin; GJB2; MUTATIONS; HEARING IMPAIRMENT; GAP-JUNCTIONS; GENE; DEAFNESS; FORM; REDUCTION; ABLATION; PATTERN; DFNB1;
D O I
10.3390/genes12071082
中图分类号
Q3 [遗传学];
学科分类号
071007 ; 090102 ;
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.
引用
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页数:15
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