COBRE: BOYS TOWN NAT RES HOSP: P4: FORMATION & REGULATION OF OTOCONIA

描述(由申请人提供)： 神经感觉障碍，特别是那些造成听力、视力和平衡/本体感觉障碍的疾病，会对个人与环境互动的能力产生毁灭性的影响，并在沟通和行动方面产生严重困难，进而影响职业和社会功能。在识别导致神经感觉障碍的基因方面取得了重要进展，但其潜在的发病机制尚不清楚。需要对神经感觉系统的发展、功能和维护进行基础研究，以了解这些疾病的分子机制，并确定最佳的干预手段。内布拉斯加州眼镜蛇的申请涉及来自内布拉斯加州大学医学中心、克雷顿大学和男童镇国家研究医院的资深研究人员和初级研究人员，他们正在结合并将他们的研究努力集中在一个共同的主题-神经感觉系统的分子生物学上。虽然该中心建立在内耳发育的现有专业知识的基础上，但它并不局限于听觉系统，允许发现感觉和外周神经系统通路的共性。提出了四个多学科研究项目，全部由初级研究人员领导，这些项目涉及神经感觉系统的发展以及影响形态发生和功能的基因/环境相互作用。核心设施允许每个项目利用各种方法，包括基因转录的测量、基因表达的本地化和创建适当的动物模型。为初级调查人员建立了指导和专业互动计划，使他们能够实现成功和独立的职业生涯。随着新项目和调查人员的增加，这一循环将永久化。其结果将是一个持续的、自我维持的和具有竞争力的研究中心，它将促进所有三个参与机构和整个州的卓越表现。

DESCRIPTION (provided by applicant): Neurosensory disorders, particularly those creating deficits in hearing, vision, and balance/proprioception have devastating effects upon the individual's ability to interact with the environment and produce significant difficulties in communication and mobility, which in turn impact occupational and social functioning. Important advances have been made in the identification of genes producing neurosensory disorders, but the underlying pathogenesis is poorly understood. Basic research into the development of neurosensory systems, their function, and their maintenance, is needed in order to understand the molecular mechanisms underlying these disorders and to determine the optimal means of intervention. This application for a Nebraska COBRE involves established researchers and junior investigators from the University of Nebraska Medical Center, Creighton University, and Boys Town National Research Hospital, who are combining and focusing their research efforts towards a common theme, the Molecular Biology of Neurosensory Systems. While this Center builds on existing expertise in the development of the inner ear, it is not restricted to the auditory system, allowing discovery of commonalities in sensory and peripheral nervous system pathways. Four multidisciplinary research projects are proposed, all led by junior investigators, which address issues of development of neurosensory systems and gene/environment interactions affecting morphogenesis and function. Core facilities allow each project to utilize various methodological approaches, including measures of gene transcription, localization of gene expression, and creation of appropriate animal models. A program of mentoring and professional interaction is established for the junior investigators, enabling them to achieve successful and independent careers. This cycle will be perpetuated by the addition of new projects and investigators. The result will be a continuing, self-sustaining, and competitive research Center that will stimulate excellence in all three participating institutions and throughout the State.