POT of gold: modeling dyskeratosis congenita in the mouse

  1. Chantal Autexier1
  1. Department of Anatomy and Cell Biology and Department of Medicine, McGill University, Montreal, Quebec H3T 1E2, Canada

Abstract

Dyskeratosis congenita (DC) is a rare syndrome, characterized by cutaneous abnormalities and premature death caused by bone marrow failure. In this issue of Genes & Development, Hockemeyer and colleagues (pp. 1773–1785) report a new mouse model that reconstitutes key features of DC. Disease phenotypes are generated by a POT1b deletion in a telomerase-deficient background that accelerates the shortening of telomeres by degradation.

Keywords

Footnotes

Related Article

  • RESEARCH PAPER: Engineered telomere degradation models dyskeratosis congenita
    • Dirk Hockemeyer,
    • Wilhelm Palm,
    • Richard C. Wang,
    • Suzana S. Couto,
    • and Titia de Lange
    Genes Dev. July 1, 2008 22: 1773-1785; Published in Advance June 11, 2008, doi:10.1101/gad.1679208
| Table of Contents

This Article

  1. doi: 10.1101/gad.1695808 Genes & Dev. 22: 1731-1736 Copyright © 2008, Cold Spring Harbor Laboratory Press

Article Category

Share

Current Issue

  1. April 1, 2024, 38 (7-8)
  1. Current Issue
  1. Alert me to new issues of G&D

Life Science Alliance