Article
Characterization of primitive hematopoietic cells from patients with dyskeratosis congenita.
Blood - 1 May 2008
Goldman Frederick D, Aubert Geraldine, Klingelhutz Al J, Hills Mark, Cooper Sarah R, Hamilton Wendy S, Schlueter Annette J, Lambie Karen, Eaves Connie J, Lansdorp Peter M
Abstract excerpt
Dyskeratosis congenita (DC) is an inherited bone marrow (BM) failure syndrome associated with mutations in telomerase genes and the acquisition of shortened telomeres in blood cells. To investigate the basis of the compromised hematopoiesis seen in DC, we analyzed cells from granulocyte colony-stimulating factor mobilized peripheral blood (mPB) collections from 5 members of a family with autosomal dominant DC...
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