CRISPR-mediated Bmpr2 point mutation exacerbates late pulmonary vasculopathy and reduces survival in rats with experimental pulmonary hypertension.
Jane Chanda KabweHirofumi SawadaYoshihide MitaniHironori OshitaNaoki TsuboyaErquan ZhangJunko MaruyamaYoshiki MiyasakaHideyoshi KoKazunobu OyaHiromasa ItoNoriko YodoyaShoichiro OtsukiHiroyuki OhashiRyuji OkamotoKaoru DohiYuhei NishimuraTomoji MashimoMasahiro HirayamaKazuo MaruyamaPublished in: Respiratory research (2022)
The present study demonstrates that the Bmpr2 mutation promotes dedifferentiation of PA smooth muscle cells, late PVD and RV myocardial fibrosis and adversely impacts both the natural and post-treatment courses of MCT-PH in rats with significant effects only in the late stages and warrants preclinical studies using this new genetic model to optimize treatment outcomes of heritable PAH.