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The neurofibromas found in NF1 patients contain not only Schwann cells, but also fibroblasts, mast cells and axons. We are currently testing each cell types’ role in neurofibroma development. For example, one project is aimed at defining the role of mast cells in a mouse transgenic model of neurofibroma formation developed in our laboratory. We are also utilizing NF1 mouse models to determine what role incomplete Schwann cell differentiation plays in NF1-associated tumor development. Another project is examining the role of neurofibroma stem and progenitor cells.
Williams JP, Wu J, Johansson G, Rizvi TA, Miller SC, Geiger H, Malik P, Li W, Mukouyama YS, Cancelas JA, Ratner N. Nf1 mutation expands an EGFR-dependent peripheral nerve progenitor that confers neurofibroma tumorigenic potential. Cell Stem Cell. Dec 4;3(6):658-69. 2008.
Wu J, Williams JP, Rizvi TA, Kordich JJ, Witte D, Meijer D, Stemmer-Rachamimov AO, Cancelas JA, Ratner N. Plexiform and dermal neurofibromas and pigmentation are caused by Nf1 loss in desert hedgehog-expressing cells. Cancer Cell. Feb;13(2):105-16. 2008.
Monk KR, Wu J, Williams JP, Finney BA, Fitzgerald ME, Filippi MD, Ratner N. Mast cells can contribute to axon-glial dissociation and fibrosis in peripheral nerve. Neuron Glia Biol. Aug;3(3):233-44. 2007.
Ling BC, Wu J, Miller SJ, Monk KR, Shamekh R, Rizvi TA, Decourten-Myers G, Vogel KS, DeClue JE, Ratner N. Role for the epidermal growth factor receptor in neurofibromatosis-related peripheral nerve tumorigenesis. Cancer Cell. Jan;7(1):65-75. 2005.
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This picture is a cross section of a human neurofibroma stained with mast cell tryptase (brown). This picture shows not only Schwann cells, fibroblasts and collagen fibers, but also degranulating mast cells. Degranulation is a symbol of mast cell activation. We are interested in identifying which cells contribute to neurofibroma formation.
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