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. 2004 Apr 30;94(8):1109-14.
doi: 10.1161/01.RES.0000126047.82846.20. Epub 2004 Mar 18.

Pulmonary hypertension in transgenic mice expressing a dominant-negative BMPRII gene in smooth muscle

James West  1 , Karen Fagan, Wolfgang Steudel, Brian Fouty, Kirk Lane, Julie Harral, Marloes Hoedt-Miller, Yuji Tada, John Ozimek, Rubin Tuder, David M Rodman
Affiliations

Pulmonary hypertension in transgenic mice expressing a dominant-negative BMPRII gene in smooth muscle

James West et al. Circ Res. .

Abstract

Bone morphogenetic peptides (BMPs), a family of cytokines critical to normal development, were recently implicated in the pathogenesis of familial pulmonary arterial hypertension. The type-II receptor (BMPRII) is required for recognition of all BMPs, and targeted deletion of BMPRII in mice results in fetal lethality before gastrulation. To overcome this limitation and study the role of BMP signaling in postnatal vascular disease, we constructed a smooth muscle-specific transgenic mouse expressing a dominant-negative BMPRII under control of the tetracycline gene switch (SM22-tet-BMPRII(delx4+) mice). When the mutation was activated after birth, mice developed increased pulmonary artery pressure, RV/LV+S ratio, and pulmonary arterial muscularization with no increase in systemic arterial pressure. Studies with SM22-tet-BMPRII(delx4+) mice support the hypothesis that loss of BMPRII signaling in smooth muscle is sufficient to produce the pulmonary hypertensive phenotype.

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