Reconstitution of the NF1 GAP-related domain in NF1-deficient human Schwann cells

Biochem Biophys Res Commun. 2006 Sep 29;348(3):971-80. doi: 10.1016/j.bbrc.2006.07.159. Epub 2006 Aug 2.

Abstract

Schwann cells derived from peripheral nerve sheath tumors from individuals with Neurofibromatosis Type 1 (NF1) are deficient for the protein neurofibromin, which contains a GAP-related domain (NF1-GRD). Neurofibromin-deficient Schwann cells have increased Ras activation, increased proliferation in response to certain growth stimuli, increased angiogenic potential, and altered cell morphology. This study examined whether expression of functional NF1-GRD can reverse the transformed phenotype of neurofibromin-deficient Schwann cells from both benign and malignant peripheral nerve sheath tumors. We reconstituted the NF1-GRD using retroviral transduction and examined the effects on cell morphology, growth potential, and angiogenic potential. NF1-GRD reconstitution resulted in morphologic changes, a 16-33% reduction in Ras activation, and a 53% decrease in proliferation in neurofibromin-deficient Schwann cells. However, NF1-GRD reconstitution was not sufficient to decrease the in vitro angiogenic potential of the cells. This study demonstrates that reconstitution of the NF1-GRD can at least partially reverse the transformation of human NF1 tumor-derived Schwann cells.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Base Sequence
  • Cell Line
  • Cells, Cultured
  • GTPase-Activating Proteins / biosynthesis
  • GTPase-Activating Proteins / deficiency*
  • GTPase-Activating Proteins / genetics*
  • GTPase-Activating Proteins / metabolism
  • Humans
  • Molecular Sequence Data
  • Neurofibromin 1 / biosynthesis
  • Neurofibromin 1 / deficiency*
  • Neurofibromin 1 / genetics*
  • Neurofibromin 1 / metabolism
  • Protein Structure, Tertiary / genetics
  • Retroviridae / genetics
  • Schwann Cells / metabolism*
  • Transduction, Genetic

Substances

  • GTPase-Activating Proteins
  • Neurofibromin 1