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NYU scientists identify critical protein complex in formation of cell cilia
Date:8/19/2008

NEW YORK, August 19, 2008 An international team led by NYU Cancer Institute have identified a protein complex that regulates the formation of cilia, which are found on virtually all mature human cells and are essential to normal cell function.

The new report, published this week by Developmental Cell and selected as the featured publication of the open-access online edition, describes how three proteins work together to regulate the formation of primary cilia. The study led by Brian Dynlacht, Ph.D., professor of pathology and director of NYU Cancer Institute Genomics Facility, investigates these antenna-like structures, once thought to be vestigial remnants of cell evolution, which have recently emerged as a focal point of research in developmental cell biology.

"We are trying to understand the regulation of processes that are fundamental to normal cell development and health in humans," said William Y. Tsang, Ph.D., of the NYU School of Medicine and Cancer Institute, and first author of the paper. "Defective cilia are implicated in a wide range of serious illnesses such as polycystic kidney disease, retinal degeneration, and neurological disorders. Inappropriate activation of signaling molecules that normally reside at the primary cilium, may lead to certain cancers."

At the center of the process lies the protein CEP290, which normally promotes primary cilia formation in mature cells. Dr. Tsang and his colleagues discovered that a second protein, CP110, normally suppresses the function of CEP290 until cells are fully mature. At that point, CP110 is destroyed, freeing CEP290 to interact with a third protein, Rab8a, to promote cilia formation on the surface of the mature cell.

The team's findings may help to identify potential targets for future drug design.

"Ciliogenesis is a fundamental process. These structures are found in almost every type of human cell you can imagine," Dr. Tsang said. "If we can e
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Contact: Jennifer Berman
Jennifer.Berman@nyumc.org
212-404-3555
NYU Langone Medical Center / New York University School of Medicine
Source:Eurekalert

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