TNF-Transgenic Mice: A new model of scleroderma pulmonary arterial hypertension

TNF-Tg mice were crossed with mice lacking either TNFR1 or TNFR2 to determine the role of TNF receptor signaling in the development of PAH and arthritis.  Assessments including clinical, histologic, imaging, and hemodynamic assessment are underway.  Preliminary studies demonstrate that TNFR2-KO/TNF-Tg are indistinguishable from TNF-Tg while TNFR1-KO/TNF-Tg are protected from pathology.