Analysis of p21-Activated Kinase Function in Neurofibromatosis Type 2

Abstract

In this project, we sought to determine if p21-activated kinases (Paks) are required for Schwann cell growth and invasiveness in cells lacking the tumor suppressor Merlin (the protein product of the neurofibromatosis-2 gene). We found that Paks are highly expressed in Schwann cells and that inhibitor of Pak - either by a peptide or by a small molecule developed by us during the course of this project - blocked proliferation and invasiveness of two different Merlin-deficient cell lines. Importantly, inhibition of Pak also blocked tumor formation in mice xenografted with Merlin-deficient cells. We also found that the beneficial effects of Pak blockade in Merlin-deficient cells were not mediated by the ERK signaling pathway. These results are important because they 1) establish Paks as potential therapeutic targets in NF2, and 2) they show that these events occur through an unexpected molecular mechanism, a problem we will continue to examine in future work.

Open PDF

Document Details

Document Type
Technical Report
Publication Date
Jan 01, 2010
Accession Number
ADA520559

Entities

People

  • Jonathan Chernoff

Tags

DTIC Thesaurus Topics

  • Cell Line
  • Cell Physiological Processes
  • Cells
  • Chemical Compounds
  • Chemical Synthesis
  • Chemistry
  • Culture Techniques
  • Cytoskeleton
  • Department Of Defense
  • Intercellular Junctions
  • Medical Personnel
  • Neoplasms
  • Peripheral Nervous System
  • Proteins
  • Small Molecules

Fields of Study

  • Biology

Readers

  • Cellular and Molecular Pathways of Apoptosis.
  • Molecular and Cellular Biology
  • Neurological Diseases/Conditions/Disorders