Individuals with NF2 are prone to the development of spinal cord tumors, called ependymomas. These glial cell tumors arise from progenitor cells in the spinal cord.
In order to develop more targeted treatments for these tumors, NF Center director, David H. Gutmann, MD, PhD, and his colleagues have employed Nf2 genetically-engineered mice to define the mechanism underlying Nf2 protein (merlin) regulation of spinal cord glial cells and progenitor cell growth. These studies have revealed new ways merlin controls cell growth in the nervous system. Current investigations are focused on merlin function in spinal cord progenitor cells in an effort to identify new therapies for NF2-associated ependymoma.
Garcia C, Gutmann DH. Nf2/merlin controls spinal cord neural progenitor function in a Rac 1/ErbB2-dependent manner. PLos One. 9: e97320, 2014.
Houshmandi SS, Emnett RJ, Giovannini M, Gutmann DH. The neurofibromatosis-2 protein, merlin, regulates glial cell growth in an ErbB2 and Src dependent manner. Mol Cell Biol. 29: 1472-86, 2009.
Lau Y-KI, Murray L, Houshmandi SS, Xu Y, Gutmann DH, Yu Q. Merlin is a potent inhibitor of glioma growth. Cancer Res. 68: 5733-42, 2008.