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Bmpr2 Mutant Rats Develop Pulmonary and Cardiac Characteristics of Pulmonary Arterial Hypertension
Author(s) -
Aurélie Hautefort,
Pedro MendesFerreira,
Jessica Sabourin,
Grégoire Manaud,
Thomas Bertero,
Catherine RückerMartin,
Marianne Riou,
Rui Adão,
Boris Manoury,
Mélanie Lambert,
Angèle Boët,
Florence Lecerf,
Valérie Domergue,
Carmen BrásSilva,
Ana M. Gómez,
David Montani,
Barbara Girerd,
Marc Humbert,
Fabrice Antigny,
Frédéric Perros
Publication year - 2019
Publication title -
circulation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 7.795
H-Index - 607
eISSN - 1524-4539
pISSN - 0009-7322
DOI - 10.1161/circulationaha.118.033744
Subject(s) - bmpr2 , medicine , pulmonary hypertension , penetrance , endocrinology , pulmonary artery , ventricle , pathology , gene , phenotype , biology , genetics , bone morphogenetic protein
Monoallelic mutations in the gene encoding bone morphogenetic protein receptor 2 ( Bmpr2) are the main genetic risk factor for heritable pulmonary arterial hypertension (PAH) with incomplete penetrance. Several Bmpr2 transgenic mice have been reported to develop mild spontaneous PAH. In this study, we examined whether rats with the Bmpr2 mutation were susceptible to developing more severe PAH.

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