HEMATOPOIESIS AND STEM CELLS Characterization of primitive hematopoietic cells from patients with dyskeratosis congenita
نویسندگان
چکیده
1Department of Pediatrics, Division of Hematology/Oncology, University of Iowa Children’s Hospital, Iowa City; 2Terry Fox Laboratory, British Columbia Cancer Agency, Vancouver, BC; 3Department of Microbiology, University of Iowa, Iowa City; 4Department of Pathology, University of Iowa, Iowa City; 5Department of Medical Genetics, University of British Columbia, Vancouver, BC; and 6Division of Hematology, Department of Medicine, University of British Columbia, Vancouver, BC
منابع مشابه
p53 Mediates Failure of Human Definitive Hematopoiesis in Dyskeratosis Congenita
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 primit...
متن کاملCharacterization of primitive hematopoietic cells from patients with dyskeratosis congenita.
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 autoso...
متن کامل"Stem cell" origin of the hematopoietic defect in dyskeratosis congenita.
We have used the long-term bone marrow culture (LTBMC) system to analyze hematopoiesis in three patients with dyskeratosis congenita (DC), two of whom had aplastic anemia, and the third had a normal blood count (apart from mild macrocytosis) and normal BM cellularity. Hematopoiesis was severely defective in all three patients, as measured by a low incidence of colony-forming cells and a low lev...
متن کاملBone marrow skeletal stem/progenitor cell defects in dyskeratosis congenita and telomere biology disorders.
Dyskeratosis congenita (DC) is an inherited multisystem disorder, characterized by oral leukoplakia, nail dystrophy, and abnormal skin pigmentation, as well as high rates of bone marrow (BM) failure, solid tumors, and other medical problems such as osteopenia. DC and telomere biology disorders (collectively referred to as TBD here) are caused by germline mutations in telomere biology genes lead...
متن کاملEffect of stem cell factor on in vitro erythropoiesis in patients with bone marrow failure syndromes.
Stem cell factor (SCF) enhances normal hematopoiesis. We examined its effect in vitro on bone marrow and blood progenitors from patients with inherited bone marrow failure syndromes, including 17 patients each with Diamond-Blackfan anemia (DBA) and Fanconi's anemia (FA), 3 with dyskeratosis congenita (DC), and 1 each with amegakaryocytic thrombocytopenia (amega) and transient erythroblastopenia...
متن کامل