Molecular Regulation of Endothelial Cells by NF-1

Abstract

We have investigated the molecular regulation pf NF1 in human endothelial cells. Using primary cell models we have generated inducible knockdown of NF1 and characterized the effects on cellular signaling, proliferation, and vascular morphogenesis. These data have been published in an appended manuscript. We have determined that mTOR signal transduction is a critical component to the NF1 response and have investigated the role of mTOR in regulating Endothelial cell proliferation and morphogenesis. We have used both siRNA and shRNA approaches to investigate the role of Ras isoforms in the NF1 mediated effects. Our data suggest that while N-Ras is the principle isoform activated upon loss of NF1, H-Ras may be more important for the manifestation of the cellular effects following NF!, particularly enhanced proliferation and altered vascular morphogenesis. Lastly we have succeeded in generating a tri-genic mouse model that allows effective inducible removal of NF1 in the vascular endothelium. Preliminary experiments indicate that the loss of NF1 in the endothelium may be sufficient to provoke endothelial cell proliferation in existing vascular beds. This should be a highly useful model of NF1 vascular disease moving forward.

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Document Details

Document Type
Technical Report
Publication Date
Jan 01, 2013
Accession Number
ADA579995

Entities

People

  • Kevin Pumiglia

Organizations

  • Albany Medical College

Tags

DTIC Thesaurus Topics

  • Arteries
  • Blood
  • Cardiovascular Diseases
  • Cardiovascular System
  • Cell Physiological Processes
  • Cells
  • Cerebrovascular Disorders
  • Congenital Abnormalities
  • Culture Techniques
  • Medical Personnel
  • Neuromuscular Diseases
  • Peptide Growth Factors
  • Vascular Diseases
  • Vascular Endothelium

Fields of Study

  • Biology

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