First Author | Autexier C | Year | 2008 |
Journal | Genes Dev | Volume | 22 |
Issue | 13 | Pages | 1731-6 |
PubMed ID | 18593874 | Mgi Jnum | J:137424 |
Mgi Id | MGI:3799548 | Doi | 10.1101/gad.1695808 |
Citation | Autexier C (2008) POT of gold: modeling dyskeratosis congenita in the mouse. Genes Dev 22(13):1731-6 |
abstractText | 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. |