Scientists ID Protein Crucial to the First Step of Cilium Formation, May Aid Understanding of Ciliopathies Like PKD

A team of researchers from Penn State University and the University of California-San Francisco has discovered a protein that is required for the growth of tiny, but critical, hair-like structures called cilia on cell surfaces. The discovery has important implications for human health because lack of cilia or problems with them can lead to serious diseases such as polycystic kidney disease PKD), blindness, and neurological disorders. "If we want to better understand and treat diseases related to cilium development, we need to identify important regulators of cilium growth and learn how those regulators function," said co-author Dr. Aimin Liu, Associate Professor of Biology at Penn State. "This work gives us significant insight into one of the earliest steps in cilium formation." The researchers describe their findings in a paper that was published online on January 27, 2014 in PNAS. In addition to Dr. Liu, article authors include Penn State cellular biologists Dr. Xuan Ye, Dr. Huiqing Zeng, and Dr. Gang Ning, as well as Dr. Jeremy F. Reiter, a biophysicist at the University of California-San Francisco. Cilia, which are present on the surface of almost all mammalian cells, are responsible for sending, receiving, and processing information within the body. "You could think of cilia as the cells' antennae," Dr. Liu said. "Without cilia, the cells can't sense what's going on around them, and they can't communicate." Cilia also perform important filtering and cleansing functions. For example, cilia inside the trachea, or windpipe, trap and prevent bacteria from entering the lungs. In a previous study, Dr. Liu and his colleagues learned that a protein called C2cd3 is important for cilium formation because mice that lacked this protein exhibited severe developmental problems typically associated with the lack of cilia.
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