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

摘要 纤毛疾病包括与引起纤毛的基因突变相关的人类疾病的不断扩大的群体 功能障碍。初级纤毛是以微管(MT)为基础的细胞器，从根尖表面突出。 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，我们对动态结构的认识仍然存在很大差距， 初级纤毛的功能，以及纤毛疾病的潜在分子基础。我们的研究项目是 重点阐明了人类CILK1致病突变的分子机制 纤毛发生相关激酶1基因导致纤毛疾病。我们的研究将对BASIC 关于初级纤毛的知识，并极大地促进了我们对疾病机制的理解 潜在的人类纤毛疾病。 Western blotting是我们实验中最常用的方法，Western blotting的质量是 对这个项目的成功至关重要。在本增刊中，我们请求购买amersham ImageQuant™800Western印迹成像系统取代过时的胶片处理机，以满足我们的 需要实现化学发光和多通道的高灵敏度和准确的定量 蛋白质印迹图上的荧光信号。

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 In this supplement, we request the purchase of Amersham ImageQuant™ 800 Western blot imaging system to replace an outdated film processor in order to meet our need to achieve high sensitivity and accurate quantification for both chemiluminescence and multi-channel fluorescence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.