Neurofibromin-deficient fibroblasts fail to form perineurium in vitro

T. Rosenbaum, Y. L. Boissy, K. Kombrinck, C. I. Brannan, N. A. Jenkins, N. G. Copeland, N. Ratner

Research output: Contribution to journalArticle

41 Scopus citations

Abstract

To identify cell type(s) that might contribute to nerve sheath tumors (neurofibromas) in patients with neurofibromatosis type 1, we generated cell cultures containing neurons, Schwann cells and fibroblasts from transgenic mouse embryos in which the type 1 neurofibromatosis gene was disrupted by homologous recombination. Normal fascicle formation by perineurial cells failed to occur in the absence of neurofibromin. Fascicles were reduced in number and showed abnormal morphology when normal neurons and Schwann cells were cultured up to 37 days with fibroblasts lacking neurofibromin. Proliferation was increased in a majority of fibroblast cell strains analyzed from embryos lacking neurofibromin. These observations suggest that mutations in the neurofibromatosis type 1 gene affect fibroblast behavior that might contribute to neurofibroma formation in patients with neurofibromatosis type 1.

Original languageEnglish (US)
Pages (from-to)3583-3592
Number of pages10
JournalDevelopment
Volume121
Issue number11
StatePublished - 1995

Keywords

  • Fibroblast
  • Neurofibroma
  • Neurofibromatosis
  • NF1
  • Perineurium
  • Ras
  • Schwann cell
  • Transgenic mice

ASJC Scopus subject areas

  • Cell Biology
  • Anatomy

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