FOXP3 and autoimmune disease: Recently it has been demonstrated that reconstitution of NSG-Ab0 DR1 mice (lacking murine MHC class II (Ab0) and expressing transgenic HLA-DR1) with HSCs from a patient with immunodysregulation polyendocrinopathy enteropathy X-linked (IPEX) syndrome, associated with FOXP3 dysfunction, spontaneously develop lethal autoimmune disease, involving multiple organs and the production of autoantibodies (245).