Cells of Origin in the Embryonic Nerve Roots for NF1-Associated Plexiform Neurofibroma

Zhiguo Chen, Chiachi Liu, Amish J. Patel, Chung Ping Liao, Yong Wang, Lu Q. Le

Research output: Contribution to journalArticle

33 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)695-706
Number of pages12
JournalCancer Cell
Volume26
Issue number5
DOIs
StatePublished - Nov 10 2014

Fingerprint

Plexiform Neurofibroma
Neurofibroma
Neoplasms
Preclinical Drug Evaluations
Inborn Genetic Diseases
Neurofibromatosis 1
Cell Lineage
Therapeutics
Population

ASJC Scopus subject areas

  • Cancer Research
  • Cell Biology
  • Oncology

Cite this

Cells of Origin in the Embryonic Nerve Roots for NF1-Associated Plexiform Neurofibroma. / Chen, Zhiguo; Liu, Chiachi; Patel, Amish J.; Liao, Chung Ping; Wang, Yong; Le, Lu Q.

In: Cancer Cell, Vol. 26, No. 5, 10.11.2014, p. 695-706.

Research output: Contribution to journalArticle

Chen, Zhiguo ; Liu, Chiachi ; Patel, Amish J. ; Liao, Chung Ping ; Wang, Yong ; Le, Lu Q. / Cells of Origin in the Embryonic Nerve Roots for NF1-Associated Plexiform Neurofibroma. In: Cancer Cell. 2014 ; Vol. 26, No. 5. pp. 695-706.
@article{700db4f8a6fb480e97bc37f33267c774,
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.}",
year = "2014",
month = "11",
day = "10",
doi = "10.1016/j.ccell.2014.09.009",
language = "English (US)",
volume = "26",
pages = "695--706",
journal = "Cancer Cell",
issn = "1535-6108",
publisher = "Cell Press",
number = "5",

}

TY - JOUR

T1 - Cells of Origin in the Embryonic Nerve Roots for NF1-Associated Plexiform Neurofibroma

AU - Chen, Zhiguo

AU - Liu, Chiachi

AU - Patel, Amish J.

AU - Liao, Chung Ping

AU - Wang, Yong

AU - Le, Lu Q.

PY - 2014/11/10

Y1 - 2014/11/10

N2 - 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.

AB - 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.

UR - http://www.scopus.com/inward/record.url?scp=84912093164&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84912093164&partnerID=8YFLogxK

U2 - 10.1016/j.ccell.2014.09.009

DO - 10.1016/j.ccell.2014.09.009

M3 - Article

C2 - 25446898

AN - SCOPUS:84912093164

VL - 26

SP - 695

EP - 706

JO - Cancer Cell

JF - Cancer Cell

SN - 1535-6108

IS - 5

ER -