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| Neurofibromas in NF1: Schwann cell origin and role of tumor environment |
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| Author(s): Zhu Y, Ghosh P, Charnay P, Burns DK, Parada LF |
| Source: SCIENCE Volume: 296 Issue: 5569 Pages: 920-922 Published: MAY 3 2002 |
| Times Cited: 168 References: 21 |
| 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. |
| Document Type: Article |
| Language: English |
| Reprint Address: Parada, LF (reprint author), Univ Texas, SW Med Ctr, Ctr Dev Biol, 6000 Harry Hines Blvd, Dallas, TX 75390 USA |
Addresses:
1. Univ Texas, SW Med Ctr, Ctr Dev Biol, Dallas, TX 75390 USA
2. Univ Texas, SW Med Ctr, Dept Pathol, Dallas, TX 75390 USA
3. Ecole Normale Super, F-75230 Paris, France |
| Publisher: AMER ASSOC ADVANCEMENT SCIENCE, 1200 NEW YORK AVE, NW, WASHINGTON, DC 20005 USA |
| Subject Category: Multidisciplinary Sciences |
| IDS Number: 549KF |
| ISSN: 0036-8075 |
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| | | | | | | | | Record from Web of Science® | | | | | | | | | |