Abstract
Abstract Neurofibromin is a product of the tumor suppressor gene Nf1 which is responsible for neurofibromatosis type 1 (NF1). When NF1 is mutated, Ras is constitutively active, enabling affected cells to proliferate extensively. This proliferation can result in tumor formation, particularly in neural crest-derived cells in the peripheral nervous system. NF1 patients can also exhibit cognitive deficits and develop other symptoms unrelated to cancer. While it is common knowledge that neurofibromin is involved in tumor formation, little is known about its function in neural stem cell proliferation and differentiation in normal and pathological settings. In addition to Ras-GAP function, recent studies have demonstrated that neurofibromin has a novel role on neuronal differentiation via its GAP-related domain. Thus we have speculated that abnormal regulation of NF1-GAP in neuronal cells may be implicated in NF1-related learning and memory disorders. The aim of the present work is to evaluate the role of neurofibromin in neuronal cell differentiation using stem cells (SCs) expressing different levels of neurofibromin. We have developed a new system to differentiate mouse SCs via formation of an embryoid body, a floating cell cluster which, by markers and function, mimicks adult neural stem cells. We have shown that the mouse embryonic ESs of all NF1 genotypes in our possession retain their pluripotency and can be differentiated into neuronal cells. This systematic study of complex molecular events in signaling pathways that occur in mouse embryonic SCs that have one (Nf1+/-), both (Nf1+/+) or neither (Nf1-/-) functional allele during in vitro differentiation is critical for a better understanding of the biology of neurofibromatosis and identification of potential targets. Citation Format: Michelle Farbaniec, Adri Chakraborty, Natalia Coleman. The role of neurofibromin in stem cell differentiation. [abstract]. In: Proceedings of the 104th Annual Meeting of the American Association for Cancer Research; 2013 Apr 6-10; Washington, DC. Philadelphia (PA): AACR; Cancer Res 2013;73(8 Suppl):Abstract nr 5054. doi:10.1158/1538-7445.AM2013-5054
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