@article{a2366cc98b4444be99eba8d38111a0c4,
title = "Cells of Origin in the Embryonic Nerve Roots for NF1-Associated Plexiform Neurofibroma",
abstract = "Neurofibromatosis type 1 is a tumor-predisposing genetic disorder. Plexiform neurofibromas are common NF1 tumors carrying a risk of malignant transformation, which is typically fatal. Little is known about mechanisms mediating initiation and identity of specific cell type that gives rise to neurofibromas. Using cell-lineage tracing, we identify a population of GAP43+ PLP+ precursors in embryonic nerve roots as the cells of origin for these tumors and report a non-germline neurofibroma model for preclinical drug screening to identify effective therapies. The identity of the tumor cell of origin and facility for isolation and expansion provides fertile ground for continued analysis to define factors critical for neurofibromagenesis. It also provides unique approaches to develop therapies to prevent neurofibroma formation in NF1 patients.",
author = "Zhiguo Chen and Chiachi Liu and Patel, {Amish J.} and Liao, {Chung Ping} and Yong Wang and Le, {Lu Q.}",
note = "Funding Information: We thank all members of the L.Q.L. lab and Dr. Luis Parada, Dr. Kristjan Jessen, Dr. Rhona Mirsky, and Dr. Wei Mo for helpful suggestions and discussions. We also thank Dr. Ueli Suter for the PlpCre-ERT mice. A.J.P. and C.-P.L. are recipients of the Young Investigator Awards from Children{\textquoteright}s Tumor Foundation. L.Q.L. holds a Career Award for Medical Scientists from the Burroughs Wellcome Fund. This work was supported by funding from the Elisabeth Reed Wagner Fund for Research and Clinical Care in Neurofibromatosis and Cardiothoracic Surgery, the Disease-Oriented Clinical Scholar Program, the National Cancer Institute of the NIH grant number R01 CA166593, and U.S. Department of Defense grant number W81XWH-12-1-0161 to L.Q.L. Publisher Copyright: {\textcopyright} 2014 Elsevier Inc.",
year = "2014",
month = nov,
day = "10",
doi = "10.1016/j.ccell.2014.09.009",
language = "English",
volume = "26",
pages = "695--706",
journal = "Cancer Cell",
issn = "1535-6108",
publisher = "Cell Press",
number = "5",
}
