Lmx1a is required for segregation of sensory epithelia and normal ear histogenesis and morphogenesis.
Lmx1a is required for segregation of sensory epithelia and normal ear histogenesis and morphogenesis.
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LMX1A是隔离感觉上皮菌和正常耳朵组织发生和形态发生所必需的。
DOI：
10.1007/s00441-008-0709-2
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发表时间：
2008-12
影响因子：
3.6
通讯作者：
Fritzsch, Bernd
中科院分区：
文献类型：
--
作者：
Nichols, David H.;Pauley, Sarah;Jahan, Israt;Beisel, Kirk W.;Millen, Kathleen J.;Fritzsch, Bernd
在 E8.5，LIM 同源域因子 Lmx1a 在整个耳基板表达，但在发育上仅限于耳朵的非感觉上皮（内淋巴管、团聚导管、耳蜗侧壁）。我们在这里确认新生的 dreher (Lmx1adr) 突变体的耳朵是畸形的。 Atoh1 和 Myo7 等毛细胞标记物首次揭示，新生的 Lmx1a 突变体只有三种感觉上皮：两个增大的耳管嵴和一个融合的上皮，包括耳蜗、球囊和椭圆囊的融合，这是一种“耳蜗重力”内器官。扩大的前耳蜗嵴由水平嵴和前嵴融合而成，而后嵴与扩大的忽略乳头融合，可能延伸到耳蜗侧壁。在融合的内器官中，耳蜗区域与前庭区域的区别在于 Gata3 等标记、盖膜的存在和耳蜗特异性神经支配。类似耳蜗的顶端显示出毛发和支持细胞的轻微紊乱。这与耳蜗区域的基底一半形成对比，耳蜗区域显示出毛细胞和支持细胞的前庭上皮样组织。耳蜗的变形特征也反映在基因表达模式的改变上。 Fgf8 表达从顶端的内毛细胞扩展到基部的大多数毛细胞。两种支持细胞标记蛋白 Sox2 和 Prox1 在基部和顶端之间的细胞分布也有所不同。 Sox2 表达在突变的耳蜗嵴扩大和融合之前先在其扩大和融合，并在耳蜗区域的基部表现出更分散和广泛的表达，而在基部中未检测到 Prox1。 Sox2 和 Prox1 表达的这些变化表明 Lmx1a 表达限制并增强 Sox2 表达，从而定义非感觉上皮和感觉上皮。成年 Lmx1a 突变 Corti 器官表现出耳蜗毛细胞的损失，表明这些突变体的长期毛细胞维持也被破坏。
At E8.5, the LIM-homeodomain factor Lmx1a is expressed throughout the otic placode but becomes developmentally restricted to non-sensory epithelia of the ear (endolymphatic duct, ductus reuniens, cochlea lateral wall). We confirm here that the ears of newborn dreher (Lmx1adr) mutants are dysmorphic. Hair cell markers such as Atoh1 and Myo7 reveal for the first time that newborn Lmx1a mutants have only three sensory epithelia: two enlarged canal cristae and one fused epithelium comprising an amalgamation of the cochlea, saccule and utricle, a “cochlear-gravistatic” endorgan. The enlarged anterior canal crista develops by fusion of horizontal and anterior crista whereas the posterior crista fuses with an enlarged papilla neglecta that may extend into the cochlear lateral wall. In the fused endorgan the cochlear region is distinguished from the vestibular region by markers such as Gata3, the presence of a tectorial membrane and cochlea-specific innervation. The cochlea-like apex displays minor disorganization of the hair and supporting cells. This contrasts with the basal half of the cochlear region which shows a vestibular epithelium-like organization of hair cells and supporting cells. The dismorphic features of the cochlea are also reflected in altered gene expression patterns. Fgf8 expression expands from inner hair cells in the apex to most hair cells in the base. Two supporting cell marker proteins, Sox2 and Prox1, also differ in their cellular distribution between the base and the apex. Sox2 expression expands in mutant canal cristae prior to their enlargement and fusion and displays a more diffuse and widespread expression in the base of the cochlear region whereas Prox1 is not detected in the base. These changes in Sox2 and Prox1 expression suggest that Lmx1a expression restricts and sharpens Sox2 expression thereby defining non-sensory and sensory epithelium. The adult Lmx1a mutant organ of Corti showed a loss of cochlear hair cells, suggesting that long term hair cell maintenance is also disrupted in these mutants.
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影响因子：
4.5
作者：
Chang, Weise;Lin, Zhengshi;Kulessa, Holger;Hebert, Jean;Hogan, Brigid L. M.;Wu, Doris K.
通讯作者：
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DOI：
10.1107/s174430910800691x
发表时间：
2008-04-01
影响因子：
0.9
作者：
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通讯作者：
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影响因子：
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作者：
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通讯作者：
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影响因子：
16.2
作者：
Bermingham, NA;Hassan, BA;Zoghbi, HY
通讯作者：
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