Bone marrow stromal cells from β-thalassemia patients have impaired hematopoietic supportive capacity.

ABSTRACT:

Background

The human bone marrow (BM) niche contains a population of mesenchymal stromal cells (MSCs) that provide physical support and regulate hematopoietic stem cell (HSC) homeostasis. β-Thalassemia (BT) is a hereditary disorder characterized by altered hemoglobin beta-chain synthesis amenable to allogeneic HSC transplantation and HSC gene therapy. Iron overload (IO) is a common complication in BT patients affecting several organs. However, data on the BM stromal compartment are scarce.

Methods

MSCs were isolated and characterized from BM aspirates of healthy donors (HDs) and BT patients. The state of IO was assessed and correlated with the presence of primitive MSCs in vitro and in vivo. Hematopoietic supportive capacity of MSCs was evaluated by transwell migration assay

SUBMITTER: Crippa S 

PROVIDER: S-EPMC6436882 | biostudies-literature | 2019 Feb

REPOSITORIES: biostudies-literature