Elimination of the fibrinogen integrin α_Mβ₂-binding motif improves renal pathology in mice with sickle cell anemia.

ABSTRACT: Sickle cell anemia (SCA) is caused by a point mutation in the β-globin gene that leads to devastating downstream consequences including chronic hemolytic anemia, episodic vascular occlusion, and cumulative organ damage resulting in death. SCA patients show coagulation activation and inflammation even in the absence of vascular occlusion. The coagulation factor fibrinogen is not only central to hemostasis but also plays important roles in pathologic inflammatory processes, in part by engaging neutrophils/macrophages through the α_Mβ₂ integrin receptor. To determine whether fibrin(ogen)-mediated inflammation is a driver of SCA-associated pathologies, hematopoietic stem cells from Berkeley sickle mice were transplanted into homozygous Fibγ^390-396A mice that exp

SUBMITTER: Nasimuzzaman M 

PROVIDER: S-EPMC6517666 | biostudies-literature | 2019 May

REPOSITORIES: biostudies-literature