Signaling Mechanisms and Cellular Functions of a Ciliopathy-associated Protein Kinase

摘要 纤毛病包括一组不断扩大的人类疾病与基因突变引起的纤毛 功能障碍初级纤毛是一个微管（MT）为基础的细胞器，从顶端表面突出， 几乎每一个哺乳动物细胞，并在化学感觉，信号转导和控制的关键作用， 各种细胞功能。到目前为止，我们对动态结构的了解仍然存在重大差距， 初级纤毛的功能和纤毛病的潜在分子基础。我们的研究项目是 集中于阐明人类CILK1致病突变的分子机制， （纤毛发生相关激酶1）基因引起纤毛病。我们的研究将对基础产生重大影响 对初级纤毛的了解，并显着推进我们对疾病机制的理解 潜在的人类纤毛病变 蛋白质印迹法是我们实验中最常用的方法，蛋白质印迹法的质量是 这对这个项目的成功至关重要。在这份补充文件中，我们要求购买阿默舍姆 ImageQuant™ 800蛋白质印迹成像系统，以取代过时的胶片处理器， 需要实现化学发光和多通道的高灵敏度和准确定量 Western印迹上的荧光信号。

Abstract Ciliopathies comprise an expanding group of human disorders associated with genetic mutations causing cilia dysfunction. The primary cilium is a microtubule (MT)-based organelle that protrudes from the apical surface of nearly every mammalian cell and plays a critical role in chemical sensation, signal transduction, and control of various cellular functions. To date, there are still major gaps in our knowledge about the dynamic structure and function of the primary cilium, and the underlying molecular basis of ciliopathies. Our research project is focused on elucidating the molecular mechanism by which pathogenic mutations in the human CILK1 (ciliogenesis associated kinase 1) gene cause ciliopathies. Our research will exert strong impact on basic knowledge about the primary cilium and significantly advance our understanding of the disease mechanisms underlying human ciliopathies. Western blotting is the most frequently used method in our experimentation and the quality of Western blots is essential for the success of this project. In this supplement, we request the purchase of Amersham ImageQuant™ 800 Western blot imaging system to replace an outdated film processor in order to meet our need to achieve high sensitivity and accurate quantification for both chemiluminescence and multi-channel fluorescence signals on Western blots.