Kinesin in photoreceptors cells

描述(由申请人提供)：动蛋白是一种微管马达蛋白，可产生许多基本细胞过程所必需的细胞内运动。该项目的长期目标是了解肌动蛋白-2在光感受器纤毛中的功能。目前的应用基于三个新的发展：(1)可以通过活细胞成像研究的纤毛中视蛋白运输的新模型；(2)将已知的视网膜疾病基因与运动蛋白-2和纤毛运输联系起来的发现；以及(3)遗传模型的改进，以便在感光细胞死亡之前研究运动蛋白-2的丢失。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说，动蛋白-2如何产生视蛋白沿纤毛运输，并测试不同的蛋白质在视蛋白沿光感受器纤毛运输中的作用。(2)确定与光感受器纤毛相关的不同(非视蛋白)转运过程中Kinesin-2的作用。这些研究的结果将有助于更好地了解光感受器纤毛中的关键细胞过程，从而为遗传性视网膜变性的主要群体提供重要的新见解。

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.