Defects in DBA: more than meets the eye.
In this issue of Blood, Garçon et al describe successful derivation of induced pluripotent stem cells (iPSs) from fibroblasts of Diamond Blackfan anemia (DBA) patients with 2 distinct ribosomal defects. Using these cells, the authors showed that they not only exhibit defective erythropoiesis but also globally impaired hematopoiesis affecting multipotent progenitors.
Hôpital Universitaire Robert-Debré.
Lydie
L
Da Costa
D029503:Anemia, Diamond-Blackfan / Q000097:blood*; D006801:Humans; D057026:Induced Pluripotent Stem Cells / Q000166:cytology*; D012270:Ribosomes / Q000378:metabolism*