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

We have recently identified that TNF-transgenic mice die prematurely of cardiopulmonary disease and done extensive work to define the major abnormality in these mice to be pulmonary hypertension.  The mice express human TNF in all cells, but it appears that non-bone marrow derived cells are driving their severe vascular phenotype.  We are performing additional studies to better characterize this model as a pre-clinical drug treatment model, to identify the mechanism by which this disease occurs, and exploring why female mice get more significant disease.