Induction of Abnormal Proliferation by Nonmyelinating Schwann Cells Triggers Neurofibroma Formation

Huarui Zheng, Lou Chang, Neha Patel, Jiong Yang, Lori Lowe, Dennis K. Burns, Yuan Zhu

Research output: Contribution to journalArticle

98 Scopus citations

Abstract

Recent evidence suggests that alterations in the self-renewal program of stem/progenitor cells can cause tumorigenesis. By utilizing genetically engineered mouse models of neurofibromatosis type 1 (NF1), we demonstrated that plexiform neurofibroma, the only benign peripheral nerve sheath tumor with potential for malignant transformation, results from Nf1 deficiency in fetal stem/progenitor cells of peripheral nerves. Surprisingly, this did not cause hyperproliferation or tumorigenesis in early postnatal period. Instead, peripheral nerve development appeared largely normal in the absence of Nf1 except for abnormal Remak bundles, the nonmyelinated axon-Schwann cell unit, identified in postnatal mutant nerves. Subsequent degeneration of abnormal Remak bundles was accompanied by initial expansion of nonmyelinating Schwann cells. We suggest abnormally differentiated Remak bundles as a cell of origin for plexiform neurofibroma.

Original languageEnglish (US)
Pages (from-to)117-128
Number of pages12
JournalCancer Cell
Volume13
Issue number2
DOIs
Publication statusPublished - Feb 5 2008

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Keywords

  • CELLCYCLE
  • STEMCELL

ASJC Scopus subject areas

  • Cancer Research
  • Cell Biology
  • Oncology

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