Marginal zone B cells are critical to factor VIII inhibitor formation in mice with hemophilia A.

ABSTRACT: Although factor VIII (FVIII) replacement therapy can be lifesaving for patients with hemophilia A, neutralizing alloantibodies to FVIII, known as inhibitors, develop in a significant number of patients and actively block FVIII activity, making bleeding difficult to control and prevent. Although a variety of downstream immune factors likely regulate inhibitor formation, the identification and subsequent targeting of key initiators in inhibitor development may provide an attractive approach to prevent inhibitor formation before amplification of the FVIII immune response occurs. As the initial steps in FVIII inhibitor development remain incompletely understood, we sought to define early regulators of FVIII inhibitor formation. Our results demonstrate that FVIII localizes in the marginal sinus

SUBMITTER: Zerra PE 

PROVIDER: S-EPMC5721282 | biostudies-literature | 2017 Dec

REPOSITORIES: biostudies-literature