Photoreceptor Ciliary Rootlet: Structure and Function

光感受器睫状根：结构和功能

• 批准号: 6914989
• 负责人: TIANSEN LI
• 金额: $ 21.24万
• 依托单位: HARVARD MEDICAL SCHOOL
• 依托单位国家: 美国
• 财政年份: 2002
• 资助国家: 美国
• 起止时间: 2002-09-30 至 2007-06-30
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/6914989
• 关键词: Escherichia coli; cytoskeletal proteins; eye; genetically modified animals; immunofluorescence technique; intermediate filaments; laboratory mouse; microfilaments; protein structure function; recombinant proteins; rod cell; tissue /cell culture; uvea ciliary body; vision; visual photoreceptor; yeast two hybrid system

DESCRIPTION (provided by applicant): The ciliary rootlet is a prominent anatomical structure of the photoreceptor but neither its molecular composition nor function is known. We have recently characterized a novel protein, rootletin, that is a component of the photoreceptor ciliary rootlet. This discovery marks a major progress in this area of study and provides the scientific framework as well as technical means to begin probing the function of the ciliary rootlet. We propose that rootletin is the major constituent of the rootlet and that rootletin polymerizes into the stable filamentous structure via its extensive coiled coil domain. Rootlet is likely to interact with a number of other cytoskeletal elements including actin filaments and intermediate filaments. These interactions may be functionally important for tensile strength, positioning of organelles, and intracellular transport. Maintenance of the normal rootlet structure is therefore likely to be essential for photoreceptor function and survival and genetic defects involving rootletin may underlie some forms of human retinal degenerative diseases. These hypotheses/questions will be addressed through a combination of in vitro and in vivo studies.

描述（由申请人提供）：纤毛根是光感受器的一个重要解剖结构，但其分子组成和功能尚不清楚。我们最近表征了一种新的蛋白质，根蛋白，这是光感受器纤毛根的一个组成部分。这一发现标志着这一研究领域的重大进展，为开始探索纤毛根的功能提供了科学框架和技术手段。我们认为根蛋白是根小的主要成分，根蛋白通过其广泛的卷曲结构域聚合成稳定的丝状结构。小根可能与许多其他细胞骨架元件相互作用，包括肌动蛋白丝和中间丝。这些相互作用可能对拉伸强度、细胞器定位和细胞内运输具有重要的功能。因此，维持正常的根茎结构可能对光感受器功能和存活至关重要，涉及根茎蛋白的遗传缺陷可能是某些形式的人类视网膜退行性疾病的基础。这些假设/问题将通过体外和体内研究的结合来解决。