Stem cell function in ß-thalassemia - HSC Cells
Hematopoietic stem cells (HSCs) are regulated by signals from the bone marrow (BM) niche, which rule hematopoiesis at steady state and in conditions associated to hematologic disorders. In severe anemia, as ß-thalassemia, which is treated by BM transplantation, an altered homeostasis has a potential impact on HSC-niche interaction and consequently on the clinical outcome. We addressed these unexplored issues in the murine disease model and in patients' cells. We report that thalassemic mice (th3) HSCs have lower frequency, reduced quiescence and impaired reconstitution potential, rescued upon transplantation in a normal BM. th3 niche results altered with defective bone deposition and osteoblast-HSC crosstalk.