Nkx5-1 controls semicircular canal formation in the mouse inner ear.
Nkx5-1 controls semicircular canal formation in the mouse inner ear.
复制标题
Nkx5-1 控制小鼠内耳半规管的形成。
作者：
Thorsten Hadrys;Thomas Braun;S. Rinkwitz;H. Arnold;E. Bober
内耳由耳囊发育而来，耳囊是一种单细胞厚的上皮，最终转化为高度复杂的结构，包括平衡感觉器官（前庭）和听力感觉器官（耳蜗）。一些具有听力和平衡缺陷的小鼠内耳突变已经被描述，但对于大多数潜在的基因尚未确定，例如，控制前庭器官发育的基因。在这里，我们报道了同源盒基因Nkx5-1在小鼠中通过同源重组失活。该基因在整个内耳发育过程中在前庭结构中表达。携带Nkx5-1零突变的小鼠表现出行为异常，类似于振动器/华尔兹型突变的典型多动和绕圈运动。平衡缺陷与Nkx5-1（-/-）突变体前庭器官的严重畸形有关，这些突变体不能发育半圆管。Nkx5-1是第一个在哺乳动物前庭系统形成中起关键作用的耳朵特异性分子。
The inner ear develops from the otic vesicle, a one-cell-thick epithelium, which eventually transforms into highly complex structures including the sensory organs for balance (vestibulum) and hearing (cochlea). Several mouse inner ear mutations with hearing and balance defects have been described but for most the underlying genes have not been identified, for example, the genes controlling the development of the vestibular organs. Here, we report the inactivation of the homeobox gene, Nkx5-1, by homologous recombination in mice. This gene is expressed in vestibular structures throughout inner ear development. Mice carrying the Nkx5-1 null mutation exhibit behavioural abnormalities that resemble the typical hyperactivity and circling movements of the shaker/waltzer type mutants. The balance defect correlates with severe malformations of the vestibular organ in Nkx5-1(-/-) mutants, which fail to develop the semicircular canals. Nkx5-1 is the first ear-specific molecule identified to play a crucial role in the formation of the mammalian vestibular system.
影响因子：
10.5
作者：
HILL, RE;JONES, PF;DAVIDSON, DR
通讯作者：
DAVIDSON, DR
Hoxa-1 突变小鼠的两个菱形体发生了改变。
DOI：
10.1242/dev.119.2.319
发表时间：
1993
期刊：
Development (Cambridge, England)
影响因子：
--
作者：
Mark,M;Lufkin,T;Vonesch,JL;Ruberte,E;Olivo,JC;Dollé,P;Gorry,P;Lumsden,A;Chambon,P
通讯作者：
Chambon,P