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Akiko Shimamura M.D. 
Funded: 07-01-2011 through 06-30-2012
Funding Type: Research Grant
Institution Location: Seattle, WA
Institution: Fred Hutchinson Cancer Research Center affiliated with Seattle Children's Hospital, University of Washington

The inherited bone marrow failure syndromes (IBMFS) are a group of disorders characterized by cancer predisposition. The study of these rare disorders has historically yielded critical insights into universal molecular pathways that cause cancer in the general pediatric population. A common feature of many of the IBMFS is impaired ribosome production or function. Ribosomes were thought to have only a housekeeping role in cells, but recent studies show that alterations in protein translation resulting from ribosomal abnormalities can promote cancer formation. There is an urgent need to better understand this connection. This project evaluates how changes in ribosome function alter protein translation to promote pediatric cancer formation.