Neurofibromas in NF1: Schwann Cell Origin and Role of Tumor Environment

Author:

Zhu Yuan1,Ghosh Pritam1,Charnay Patrick2,Burns Dennis K.3,Parada Luis F.1

Affiliation:

1. Center for Developmental Biology,

2. Ecole Normale Superieure, 46 rue d'Ulm 75230, Paris, Cedex 05, France.

3. Department of Pathology, University of Texas Southwestern Medical Center, 6000 Harry Hines Boulevard, Dallas, TX 75390–9133, USA.

Abstract

Neurofibromatosis type 1 (NF1) is one of the most prevalent dominantly inherited genetic diseases of the nervous system. NF1 encodes a tumor suppressor whose functional loss results in the development of benign neurofibromas that can progress to malignancy. Neurofibromas are complex tumors composed of axonal processes, Schwann cells, fibroblasts, perineurial cells, and mast cells. Through use of a conditional ( cre / lox ) allele, we show that loss of NF1 in the Schwann cell lineage is sufficient to generate tumors. In addition, complete NF1-mediated tumorigenicity requires both a loss of NF1 in cells destined to become neoplastic as well as heterozygosity in non-neoplastic cells. The requirement for a permissive haploinsufficient environment to allow tumorigenesis may have therapeutic implications for NF1 and other familial cancers.

Publisher

American Association for the Advancement of Science (AAAS)

Subject

Multidisciplinary

Reference23 articles.

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3. Zhu Y., Parada L. F., Exp. Cell Res. 264, 19 (2001).

4. Ballester R., et al., Cell 63, 851 (1990).

5. Xu G. F., et al., Cell 63, 835 (1990).

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