Modeling Phenotypes of Tuberous Scerosis in the Mouse

Abstract

The overall goal of this project is to generate a mouse model of the smooth muscle-related facets of tuberous sclerosis, specifically in an attempt to model the lung phenotype seen in a subset of TS patients and patients with LAM. We have conditionally targeted the TSC1 gene in smooth muscle, which results in mortality at approximately 10 weeks of age. This mouse now provides a useful model in which to investigate the function of individual MMPs or other proteins in this pathological progression, and to evaluate relevant therapeutic interventions such as rapamycin.

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Document Details

Document Type
Technical Report
Publication Date
Feb 01, 2007
Accession Number
ADA466831

Entities

People

  • James M. Shipley

Organizations

  • Washington University in St. Louis

Tags

DTIC Thesaurus Topics

  • Biomedical Research
  • Breeding
  • Cells
  • Culture Techniques
  • Genes
  • Genetic Phenomena
  • Genetics
  • Health Services
  • Intervention
  • Lung Diseases
  • Muscle Cells
  • Muscles
  • Phenotypes
  • Proteins
  • Sclerosis
  • Smooth Muscle
  • Tissues

Readers

  • Aquatic Ecology
  • Computational Modeling and Simulation
  • Immunology and Pathology