Article
A large animal model of heritable pulmonary arterial hypertension using BMPR2 gene-edited sheep.
JCI insight - 24 Aug 2026
Datar Sanjeev A, Werry Nicholas, Brown Austin R, Fitzpatrick Devon S, Falade Oluwafemi, Trott Josephine F, Hutchings Rachel, Amin Elena K, Morgan Jessica M, Nawaytou Hythem, Deutsch Gail H, Johnson Eric G, Gonzales Viera Omar A, Bishop Thomas F, Beach Tara Urbano, McNabb Bret R, Austin Eric D, Fineman Jeffery R, Van Eenennaam Alison L
Abstract excerpt
Pulmonary arterial hypertension (PAH) is a rare vascular disorder characterized by elevated pressure in pulmonary arteries, eventually leading to right ventricular failure. Approximately 50% of pediatric disease and 20% of adult disease can be linked to a genetic mutation, with nearly 70% of these cases involving mutations in the bone morphogenetic protein receptor type 2 (BMPR2) locus. Investigations using...
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