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Researchers identify mechanism, possible drug treatment for tumors in neurofibromatosis
Date:10/30/2008

Researchers studying neurofibromatosis type 1 a rare disease in which tumors grow within nerves have found that the tumors are triggered by crosstalk between cells in the nerves and cells in the blood. The researchers, who were funded by the National Institutes of Health (NIH) and the Department of Defense (DOD), also found that a drug on the market for treating certain kinds of blood cancer curbs tumor growth in a mouse model of neurofibromatosis type 1. A clinical trial of the drug is underway in people with the disease.

The results of the study on mice are published in the October 31, 2008 issue of Cell.

The study's senior investigators were Luis F. Parada, Ph.D., a neuroscientist at the University of Texas Southwestern Medical Center in Dallas, and D. Wade Clapp, M.D., a hematologist at the Indiana University School of Medicine in Indianapolis. Their research was supported by NIH's National Institute of Neurological Disorders and Stroke (NINDS), NIH's National Cancer Institute (NCI), and the Neurofibromatosis Research Program of the U.S. Army Medical Research and Material Command.

"By taking a team approach and combining their unique areas of expertise, Drs. Parada and Clapp were able to shed light on a complicated disease mechanism and to develop a potential treatment," says Jane Fountain, Ph.D., a program director with NINDS.

Neurofibromatosis type 1 is a genetic disease that affects about 1 in 3500 Americans. The nerve-associated tumors, or neurofibromas, that occur in the disease tend to grow just under the skin or at the nerve root. The latter type of tumor, called a plexiform neurofibroma, can cause disabling symptoms by compressing the nerve, the spinal cord, bones, muscles and internal organs.

The tumor forming cells within a neurofibroma may become malignant and spread to other parts of the body. There is currently no treatment to prevent neurofibroma growth.

In 1990, NIH-funded i
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Contact: Daniel Stimson
stimsond@ninds.nih.gov
301-496-5751
NIH/National Institute of Neurological Disorders and Stroke
Source:Eurekalert

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