Shen Yu-Chi, Jeyabalan Anandhi K, Wu Karen L, Hunker Kristina L, Kohrman David C, Thompson Deborah L, Liu Dong, Barald Kate F
Department of Cell and Developmental Biology, University of Michigan Medical School, Ann Arbor, Michigan 48109-2200, USA.
Dev Dyn. 2008 Apr;237(4):941-52. doi: 10.1002/dvdy.21486.
The inner ear is a complex organ containing sensory tissue, including hair cells, the development of which is not well understood. Our long-term goal is to discover genes critical for the correct formation and function of the inner ear and its sensory tissue. A novel gene, transmembrane inner ear (Tmie), was found to cause hearing-related disorders when defective in mice and humans. A homologous tmie gene in zebrafish was cloned and its expression characterized between 24 and 51 hours post-fertilization. Embryos injected with morpholinos (MO) directed against tmie exhibited circling swimming behavior (approximately 37%), phenocopying mice with Tmie mutations; semicircular canal formation was disrupted, hair cell numbers were reduced, and maturation of electrically active lateral line neuromasts was delayed. As in the mouse, tmie appears to be required for inner ear development and function in the zebrafish and for hair cell maturation in the vestibular and lateral line systems as well.
内耳是一个复杂的器官,包含感觉组织,其中包括毛细胞,但其发育过程尚未完全明晰。我们的长期目标是发现对于内耳及其感觉组织的正确形成和功能至关重要的基因。发现了一个新基因——跨膜内耳(Tmie),当该基因在小鼠和人类中出现缺陷时,会引发与听力相关的疾病。克隆了斑马鱼中的同源tmie基因,并对其在受精后24至51小时之间的表达进行了表征。注射针对tmie的吗啉代寡核苷酸(MO)的胚胎表现出转圈游泳行为(约37%),模拟了具有Tmie突变的小鼠;半规管形成受到破坏,毛细胞数量减少,电活性侧线神经丘的成熟延迟。与小鼠一样,tmie似乎是斑马鱼内耳发育和功能以及前庭和侧线系统中毛细胞成熟所必需的。