Kinesin in photoreceptors cells

描述（由申请人提供）：运动蛋白是微管运动蛋白，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激酶-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)新建立的纤毛视蛋白运输模型，可以通过活细胞成像研究；(2)将已知视网膜疾病基因与驱动蛋白-2和睫状体运输联系起来的发现；(3)改进遗传模型，以便在光感受器细胞死亡之前研究驱动蛋白-2的损失。我们建议利用这些新发现和发展：(1)测试两个相互竞争的假设，关于驱动蛋白-2如何沿纤毛产生视蛋白运输，并测试不同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）运输过程中激酶2的贡献。这些研究结果将有助于更好地理解光感受器纤毛的关键细胞过程，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.