Cell of origin and the contribution of microenvironment in NF1 tumorigenesis and therapeutic implications

Johanna Buchstaller, D. Clapp, Luis F. Parada, Yuan Zhu

Research output: Chapter in Book/Report/Conference proceedingChapter

2 Scopus citations

Abstract

The hallmark feature of neurofibromatosis type 1 (NF1) is the development of multiple benign peripheral nerve sheath tumors (PNST), also known as neurofibroma. One of the major clinical consequences of germline NF1 mutations in patients is the conversion of a relatively tumor-resistant organ, the peripheral nerve, to a highly tumor-susceptible organ. However, it is not clear about the molecular and cellular mechanism(s) by which NF1 heterozygous mutations in the germline alter nerve cell biology during development and disrupt nerve microenvironment, thereby promoting tumor development. At least some of the answers to these fundamental questions lie in the identification of the cell(s) of origin of these benign and malignant peripheral nerve sheath tumors. This review summarizes the recent progress of the cell-of-origin research on NF1-associated tumors, which has provided important insights into the design of therapies for preventing and treating these incurable human tumors.

Original languageEnglish (US)
Title of host publicationNeurofibromatosis Type 1: Molecular and Cellular Biology
PublisherSpringer-Verlag Berlin Heidelberg
Pages549-568
Number of pages20
Volume9783642328640
ISBN (Print)9783642328640, 3642328636, 9783642328633
DOIs
StatePublished - Aug 1 2012

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ASJC Scopus subject areas

  • Medicine(all)
  • Biochemistry, Genetics and Molecular Biology(all)

Cite this

Buchstaller, J., Clapp, D., Parada, L. F., & Zhu, Y. (2012). Cell of origin and the contribution of microenvironment in NF1 tumorigenesis and therapeutic implications. In Neurofibromatosis Type 1: Molecular and Cellular Biology (Vol. 9783642328640, pp. 549-568). Springer-Verlag Berlin Heidelberg. https://doi.org/10.1007/978-3-642-32864-0_36