Kinesin in photoreceptors cells

描述（由申请人提供）：驱动蛋白是微管马达蛋白，产生许多基本细胞过程所必需的细胞内运动。本项目的长期目标是了解驱动蛋白-2在光感受器纤毛中的功能。目前的应用是基于三个新的发展：（1）一个新建立的模型，视蛋白运输的纤毛，可以通过活细胞成像研究;（2）的发现，已知的视网膜疾病基因链接驱动蛋白-2和纤毛运输;（3）完善的遗传模型，使损失的驱动蛋白-2可以研究感光细胞死亡之前。我们建议利用这些新的发现和发展：（1）测试两个相互竞争的假设，驱动蛋白-2如何产生视蛋白运输沿着纤毛，并测试不同的蛋白质的作用，在运输的视蛋白沿着感光纤毛。(2)确定驱动蛋白-2在与感光器纤毛相关的不同（非视蛋白）运输过程中的贡献。这些研究的结果将导致更好地了解感光器纤毛中的关键细胞过程，从而为一组主要的遗传性视网膜变性提供重要的新见解。

DESCRIPTION (provided by applicant): Kinesins are microtubule motor proteins that generate intracellular movement essential for many fundamental cellular processes. The long-term goal of this project is to understand the function of kinesin-2 in the photoreceptor cilium. The current application is based on three new developments: (1) A newly established model of opsin transport in cilia that can be studied by live-cell imaging; (2) Findings that link known retinal disease genes to kinesin-2 and ciliary transport; and (3) Refinement of the genetic model so that loss of kinesin-2 can be studied prior to photoreceptor cell death. We propose to capitalize upon these new findings and developments by: (1) Testing two competing hypotheses for how kinesin-2 generates opsin transport along the cilium, and testing the roles of different proteins in the transport of opsin along the photoreceptor cilium. (2) Determining the contribution of kinesin-2 in different (non-opsin) transport processes related to the photoreceptor cilium. The results of these studies will lead to a better understanding of critical cellular processes in the photoreceptor cilium, and thus provide important new insight into a major group of inherited retinal degenerations.