Model found to determine cause, possible treatment of ne...( CINCINNATI A multi-institutional st...),Health

CINCINNATI A multi-institutional study led by researchers at Cincinnati Childrens Hospital Medical Center has led to new insights and a model to help unravel the cause of fibrous, non-cancerous nerve tumors called neurofibromas, which can lead to disfigurement and in rare cases death by compressing vital organs. Writing for the February Cancer Cell, researchers said their findings also provide a means for testing therapies that could eventually help patients who now have limited options for treating the disease, called Neurofibromatosis type 1.

We have developed a mouse model that allows us to mimic as closely as possible the formation in humans of two kinds of neurofibromas, which are non-cancerous tumors that affect children and adults, said Nancy Ratner, Ph.D., study co-author and a researcher in the Division of Experimental Hematology and Cancer Biology at Cincinnati Childrens. Our findings give us a way to begin clarifying the biological mechanisms underlying the tumor formation in Neurofibromatosis and to have a platform for therapeutic testing.

Previous research has determined that neurofibromas result from a mutation in a gene (called Nf1), which normally acts as a tumor suppressor by producing a complex protein called neurofibromin to help regulate cell growth. Mutation of the Nf1 gene inhibits neurofibromin production, leading to irregular cell growth and possible tumor development. Dr. Ratner and her colleagues suggest in their study that the timing of this gene mutation is critical to whether neurofibroma tumors form.

In one set of experiments, the researchers worked with genetically engineered nerve root cells from embryonic mice. They discovered that mutating both of two mouse Nf1 genes at 12.5 days after conception (a critical stage in mouse embryonic development when cells that eventually differentiate to form the nervous system are just getting ready to do so) would create cell colonies. In another set of experiments, researc
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