POT of gold: modeling dyskeratosis congenita in the mouse

被引：6

作者：

Autexier, Chantal ^{[1
,2
]}

机构：

[1] McGill Univ, Dept Anat & Cell Biol, Montreal, PQ H3T 1E2, Canada

[2] McGill Univ, Dept Med, Montreal, PQ H3T 1E2, Canada

来源：

GENES & DEVELOPMENT | 2008年 / 22卷 / 13期

关键词：

bone marrow failure; dyskeratosis congenita; POT1; shelterin; telomere; telomerase;

D O I：

10.1101/gad.1695808

中图分类号：

Q2 [细胞生物学];

学科分类号：

071009 ; 090102 ;

摘要：

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.

引用

页码：1731 / 1736

页数：6

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