Mononuclear cells and vascular repair in HHT

摘要: Hereditary hemorrhagic telangiectasia (HHT) or Rendu-Osler-Weber disease is a rare genetic vascular disorder known for its endothelial dysplasia causing arteriovenous malformations and severe bleedings. HHT-1 HHT-2 are the most prevalent variants caused by heterozygous mutations in endoglin ALK1, respectively. An undervalued aspect of that HHT patients experience persistent inflammation. Although mural cells have been main research focus trying to unravel mechanism behind disease, wound healing process with delicate balance between inflammatory cells. Inflammatory part mononuclear (MNCs) fraction, can, next eliciting an immune response, also angiogenic potential. This biphasic effect MNC can hold promising further elucidate treatment strategies patients. Before able contribute repair, they need home retain ischemic damaged tissue. Directed migration (homing) following tissue damage regulated stromal cell derived factor 1 (SDF1). MNCs express C-X-C chemokine receptor 4 (CXCR4) migrate towards tightly gradient SDF1. directed monocytes lymphocytes be inhibited dipeptidyl peptidase (DPP4). Interestingly, elevated levels DPP4 show impaired homing Impaired capacity might therefore angiogenesis repair observed review summarizes recent studies regarding role etiology evaluates efficacy inhibition integrity repair.