A team of Massachusetts General Hospital (MGH) Cancer Center researchers has determined one way that mutations in a gene involved in a rare, hereditary cancer syndrome lead to out-of-control cellular proliferation. In their report published online in Genes & Development, the team describes finding how mutations in the NF2 (neurofibromatosis type 2) gene, which codes for the merlin protein, make cells hyper-responsive to growth factor signaling. In addition to neurofibromatosis type 2, a genetic disorder that induces the formation of numerous benign tumors throughout the nervous system, NF2 mutations have also been associated with the dangerous malignant cancer mesothelioma.
“We have found that NF2-mutant cells of multiple types – including cells that initiate nervous system tumors in familial NF2 and mesothelioma cells with the NF2 mutation – excel at a natural process cells use to gulp up and internalize fluids from their environment, as if they were captured by a breaking wave,” says Andrea McClatchey, PhD, of the MGH Center for Cancer Research, senior author of the paper. “This ability enables cells to respond excitedly to growth factors they normally might not detect and may allow NF2-mutant cells to survive and grow outside their normal milieu, ingesting extra nutrients
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