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

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 In this supplement, we request the purchase of Amersham ImageQuant™ 800 Western blot imaging system to replace an outdated film processor in order to meet our need to achieve high sensitivity and accurate quantification for both chemiluminescence and multi-channel fluorescence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