Clapper, Patti, Schweizer and Mason

Patients suffering from a rare inherited cancerous tumor disorder called Neurofibromatosis Type 2 (or NF2) are the subject of a new study which could provide a measure of hope for mesothelioma patients as well. 

The disease, sometimes called MISME Syndrome (Multiple Inherited Schwannomas, Meningiomas, and Ependymomas), is caused by a mutation in both copies of the NF2 gene.  The condition affects roughly one in 30,000 people and causes sufferers to develop non-malignant brain tumors which impinge upon nerves that serve as conduits for vision and hearing. 

In research published in the April 12 issue of Cancer Cell, Wistar Institute investigators propose a radical new type of treatment for the disease.  Instead of attacking the cancerous cells head on, scientists looked at what would happen to these tumors if they attacked the proteins which provide cells with “personal space.”

The NF2 gene creates a protein product named Merlin which signals cells to stop growing when they come into close proximity to other cells.  Merlin binds to the protein angiomotin.  When this occurs, the cell is “ordered” to stop growing.

Experiments to find out exactly how Merlin interacts with angiomotin have led to an important discovery—a better understanding of the relationship between chemical “sensors” that detect interactions between cells and how those sensor trigger cell division.

Joseph Kissil, Ph.D., said in a statement that he was very excited by this line of research because "Angiomotin is required for movement of cells that form new blood vessels, so it is fascinating to see it so closely linked to Merlin … loss of which leads to tumor formation." Kissil, who is an associate professor in the Molecular and Cellular Oncogenesis Program of The Wistar Institute Cancer Center, went on to describe that this new revelation may allow researchers to construct a treatment that would effectively starve tumor cells, preventing them from growing any larger and perhaps even shrinking them in the process.

The Wistar Institute’s scientists were targeting NF2 but the research could theoretically be applied to mesothelioma patients as well.  Mesothelioma tumors caused by exposure to asbestos fibers shares some similar properties to NF2 tumors.  (Merlin mutations are present in roughly 50% of all mesothelioma cases.) 

While Kissil plans to continue with experiments centering on NF2, he is anxious to explore how this new data could possibly enhance treatments for other types of cancer such as mesothelioma and plans to pursue that line of experimentation in the near future.