Utah researchers have shown that the gene that causes neurofibromatosis belongs to a family of genes that suppress tumor formation. The new discovery, reported last week in the journal Cell, indicates that research on the gene will provide not only information useful for treating neurofibromatosis but also new insights into the mechanisms by which cancer occurs.
Neurofibromatosis is the most common nervous system defect caused by a single gene defect. It affects one out of every 3,500 people, causing symptoms that range from brownish skin lesions to massive, deforming tumors called neurofibromas, seizures and fatal tumors of the nervous system.
The identification and cloning of the gene that causes the disorder was reported barely three weeks ago. "We didn't expect to have the protein produced by the gene for another 12 to 24 months," said Peter Bellerman of the National Neurofibromatosis Foundation. "Now we have it. It's a tremendous leap forward."
Geneticist Ray White and his colleagues at the University of Utah found that the protein produced by the gene suppresses the activity of certain oncogenes--naturally occurring genes that lead to cancer. Knowing its identity, he predicted, would allow researchers to synthesize chemicals that could both block tumor formation and prevent the growth of neurofibromas.