Abstract:Hereditary hemorrhagic telangiectasia type 1 (HHT1) is a severe vascular disorder caused by mutations in the TGFβ/BMP co-receptor endoglin. Endoglin haploinsufficiency results in vascular malformations and impaired neoangiogenesis. Furthermore, HHT1 patients display an impaired immune response. To date it is not fully understood how endoglin haploinsufficient immune cells contribute to HHT1 pathology. Therefore, we investigated the immune response during tissue repair in Eng+/− mice, a model for HHT1. Eng+/− m… Show more
“…These data somehow reflect data from the clinic, as patients with HHT1 also show an increased number of inflammatory macrophages. Astonishingly, the treatment of Eng+/− mice with a bone morphogenetic protein (BMP) receptor kinase inhibitor improved heart function and vascularization, suggesting that the BMP receptor kinase may present a promising therapeutic target for HHT1 patients in the future [16].…”
Vascular occlusive diseases such myocardial infarction, peripheral artery disease of the lower extremities, or stroke still represent a substantial health burden worldwide [...]
“…These data somehow reflect data from the clinic, as patients with HHT1 also show an increased number of inflammatory macrophages. Astonishingly, the treatment of Eng+/− mice with a bone morphogenetic protein (BMP) receptor kinase inhibitor improved heart function and vascularization, suggesting that the BMP receptor kinase may present a promising therapeutic target for HHT1 patients in the future [16].…”
Vascular occlusive diseases such myocardial infarction, peripheral artery disease of the lower extremities, or stroke still represent a substantial health burden worldwide [...]
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