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Neurofibromatosis type 1 — a model for nervous system tumour formation?

Nature Reviews Cancer volume 5, pages 557–564 (2005)Cite this article

Abstract

Neurofibromatosis type 1 (NF1) is a common genetic condition in which affected individuals develop benign and malignant nervous system tumours. Genetically engineered mouse (GEM) models of these NF1-associated nervous system tumours recapitulate several of the unique clinical aspects of the disease. Moreover, these Nf1 GEM models allow for a direct examination of the earliest stages of tumour evolution, including the contributions that Nf1+/− cellular elements and cooperating genetic changes make to facilitate the transition from the pre-neoplastic to the neoplastic state and, in some cases, to promote malignant progression.

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Figure 1: Structure of the NF1 gene product, neurofibromin.
Figure 2: Signalling pathways that are regulated by neurofibromin.
Figure 3: Evolution of NF1-associated peripheral and central nervous system tumours.
Figure 4: Interaction between stromal cells and hyperproliferating pre-neoplastic cells.

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Acknowledgements

The authors thank our Neurofibromatosis Center Group at Washington University, especially A. Perry, J. Weber, M. Watson and J. Garbow, as well as R. Wechsler-Reya for critical reading of the manuscript. D.H.G. is supported by grants from the Department of Defense, National Institute of Neurological Disorders and Strokes, and James S. McDonnell Foundation. J.R. is a scholar of the Child Health Research Center of Excellence in Developmental Biology at Washington University School of Medicine and receives additional support from the National Institute of Child Health and Human Development, The American Cancer Society and Hope Street Kids. We also acknowledge the generous support from Schnuck Markets, Inc.

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  1. the Departments of Pediatrics and Anatomy and Neurobiology, Washington University School of Medicine and St. Louis Children's Hospital, St. Louis, 63110, Missouri, USA

    Joshua B. Rubin

  2. the Department of Neurology, Washington University School of Medicine and St. Louis Children's Hospital, St. Louis, 63110, Missouri, USA

    David H. Gutmann

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Correspondence to David H. Gutmann.

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DATABASES

Entrez Gene

CDKN2A

NF1

National Cancer Institute

leukaemia

pheochromocytoma

OMIM

neurofibromatosis type I

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Gutmann lab

Rubin lab

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Rubin, J., Gutmann, D. Neurofibromatosis type 1 — a model for nervous system tumour formation?. Nat Rev Cancer 5, 557–564 (2005). https://doi.org/10.1038/nrc1653

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