Over-expression of myosin7A in cochlear hair cells of circling mice.
Over-expression of myosin7A in cochlear hair cells of circling mice.
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DOI：
10.5625/lar.2017.33.1.1
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发表时间：
2017-03
影响因子：
2.9
通讯作者：
Suh JG
中科院分区：
文献类型：
--
作者：
Kim YY;Nam H;Jung H;Kim B;Suh JG
删除跨膜内耳（Tmie）基因的Circling小鼠（C57BL/6J-cir/cir）是人类非综合征性隐性耳聋DFNB6的动物模型。在旋转的小鼠中，随着时间的推移，耳蜗中的毛细胞已经退化，毛束变得不规则。 Tmie 蛋白在耳蜗毛细胞中发挥机电转导通道的功能。肌球蛋白 7a (MYO7A) 蛋白在耳蜗毛束的发育以及耳蜗毛细胞的功能中发挥着关键作用。为了确定 Tmie 蛋白在出生后发育阶段是否与 MYO7A 蛋白相互作用，我们通过蛋白质印迹分析和出生后第 5 天（P5）的整体免疫荧光研究了绕圈小鼠耳蜗毛细胞中 MYO7A 蛋白的表达。 C57BL/6J-+/cir 和 C57BL/6J-cir/cir 小鼠耳蜗中 MYO7A 的表达较 C57BL/6J-+/+ 小鼠显着增加。与 C57BL/6J-+/+ 小鼠相比，C57BL/6J-+/cir 和 C57BL/6J-cir/cir 小鼠耳蜗毛细胞的 MYO7A 强度也有所增加。综上所述，结果表明，Tmie 蛋白可能与 MYO7A 蛋白一起在耳蜗出生后发育阶段的静纤毛束的发育和维持中发挥重要作用。
Circling mouse (C57BL/6J-cir/cir) deleted the transmembrane inner ear (Tmie) gene is an animal model for human non-syndromic recessive deafness, DFNB6. In circling mouse, hair cells in the cochlea have degenerated and hair bundles have become irregularity as time goes on. Tmie protein carries out a function of the mechanoelectrical transduction channel in cochlear hair cells. Myosin7a (MYO7A) protein has key roles in development of the cochlear hair bundles as well as in the function of cochlear hair cells. To find whether Tmie protein interacts with MYO7A proteins in the cochlea postnatal developmental stage, we investigated expression of the MYO7A proteins in the cochlear hair cells of circling mice by western blot analysis and whole mount immunofluorescence at postnatal day 5 (P5). The expression of MYO7A showed statistically significant increase in the cochlea of C57BL/6J-+/cir and C57BL/6J-cir/cir mice than that of C57BL/6J-+/+ mice. The MYO7A intensity of the cochlear hair cells also increased in C57BL/6J-+/cir and C57BL/6J-cir/cir mice compared with those of C57BL/6J-+/+ mice. Taken together, the results indicate that Tmie protein may have an important role with MYO7A protein in the development and maintenance of the stereociliary bundles during postnatal developmental stage of the cochlea.