Transcription profiling of mouse inducible BMPR2 mutant vs. controls in lung with and without elevated RVSP

ABSTRACT: Familial pulmonary arterial hypertension (fPAH) is associated with mutations in BMPR2. Many of these mutations occur in the BMPR2 tail domain, leaving the SMAD functions intact. In order to determine the in vivo consequences of BMPR2 tail domain mutation, we created a smooth-muscle specific doxycycline inducible BMPR2 mutation with an arginine to termination mutation at amino acid 899. When these SM22-rtTA x TetO7-BMPR2R899X mice had transgene induced for 9 weeks, starting at 4 weeks of age, they universally developed pulmonary vascular pruning as assessed by fluorescent microangiography. Approximately half the time the induced animals developed elevated right ventricular systolic pressures (RVSP), associated with extensive pruning, muscularization of small pulmonary vessels, and developme

ORGANISM(S): Mus musculus

SUBMITTER: James West 

PROVIDER: E-GEOD-11018 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress