Article
p53 Mediates Failure of Human Definitive Hematopoiesis in Dyskeratosis Congenita.
Stem cell reports - 8 Aug 2017
Fok Wilson Chun, Niero Evandro Luis de Oliveira, Dege Carissa, Brenner Kirsten Ann, Sturgeon Christopher Michael, Batista Luis Francisco Zirnberger
Abstract excerpt
Dyskeratosis congenita (DC) is a bone marrow failure syndrome associated with telomere dysfunction. The progression and molecular determinants of hematopoietic failure in DC remain poorly understood. Here, we use the directed differentiation of human embryonic stem cells harboring clinically relevant mutations in telomerase to understand the consequences of DC-associated mutations on the primitive and definitive...
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