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首页> 外文期刊>Experimental Animals >Function of Myosin VI in wild type and mutant mice during inner ear hair cell development
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Function of Myosin VI in wild type and mutant mice during inner ear hair cell development

机译：肌球蛋白VI在野生型和突变型小鼠内耳毛细胞发育过程中的功能

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摘要

Mutations in the gene encoding the unconventional myosin, MyosinVI (Myo6), cause non-syndoromic deafness in human or Snell's waltzer (Myo6~(sv)) mutation in mice. The Myo6~(sv/sv) stereocilia are fused at the bases. We have characterized a new mutant allele of the Myo6 gene that arose as a spontaneous, recessive mutation in the C57BL/6J, and the mutation is associated with chromosomal inversion on chromosome 9. We have investigated the function of Myo6 in the inner hair cells using this new Myo6 mutant. Morphologic study revealed normal to moderateleary defective cochlear hair cell stereocilia in this mutant at new born mice (postnatal day 0; P0). At the stage, we also observed some irregular orientation of stereocilia bundles and distorted cuticular plates. Stereocilia abnormalities were consistently present P5 and P10. Degenerative changes including loss of inner and outer hair cells were seen at P30. We analyzed the expression of MY06 in the hair cells for developmental stages between late embryonic and postnatal stage. MYO6 in the developing mouse cochlear hair cells was first detected in the cuticular plates of inner hair cells at El 5.5, and the immunoactivity was consistently present at the cuticular plates in the cochlear hair cells during mouse postnatal development and in the adult. A similar distribution of MY06 staining was observed at the cuticular plates of vestibular hair cells. As other MYO6 localization, the brightest staining observed at the pericuticular recklace, and the labeling increase with the progress of development. These observations suggest two possible rules for MY06 in hair cell: maintenance of the stereocilia and cuticular plates, and anchoring stereocilia rootlets.
机译：编码非常规肌球蛋白MyosinVI（Myo6）的基因中的突变会导致人的非综合征性耳聋或小鼠的Snell's Waltzer（Myo6〜（sv））突变。 Myo6〜（sv / sv）立体纤毛在基部融合。我们已经表征了一个新的Myo6基因突变等位基因，它在C57BL / 6J中作为自发的，隐性突变出现，并且该突变与9号染色体上的染色体倒置有关。我们已经研究了Myo6在内部毛细胞中的功能这个新的Myo6突变体。形态学研究显示，在新生小鼠中，该突变体的正常至中等缺陷性耳蜗毛细胞立体纤毛（出生后第0天； P0）。在这个阶段，我们还观察到了一些纤毛束和不规则的角质层板不规则的取向。一直存在P5和P10的立体视觉异常。在P30观察到变性变化，包括内部和外部毛细胞的损失。我们分析了从胚胎后期到出生后的发育阶段，毛细胞中MY06的表达。首先在El 5.5处在内部毛细胞的角质层板中检测到发育中的小鼠耳蜗毛细胞中的MYO6，并且在小鼠出生后和成年期中，在耳蜗毛细胞的角质层板中始终存在免疫活性。在前庭毛细胞的表皮板上观察到MY06染色的类似分布。与其他MYO6定位一样，在表皮周围的斑点上观察到最亮的染色，并且标记随着显影的进行而增加。这些观察结果提示毛细胞MY06有两个可能的规则：维持纤毛和表皮板，以及锚定纤毛根。

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