UIC-2012-049 – A transgenic mouse model of pulmonary arterial hypertension (PAH).

Over a quarter of a million individuals a year are hospitalized for pulmonary arterial hypertension (PAH). Survival rates of these patients are typically less than 3 years post diagnosis and there is currently no cure or tractable animal model for PAH. Researchers in Pediatrics at UIC have developed an animal model by crossing two strains of mice with conditional transgenes. Mice expressing floxed Von Hippel-Lindau protein (VHL) from Jackson Labs (B6.129S4(C)-Vhltm1Jae/J) were bred with animals expressing a fibroblast specific promotor driving Cre (Fsp-Cre) obtained from Ohio State University. Resulting offspring Fsp-VHL+/- and Fsp-VHL-/- were completely viable and fertile. By three months of age these mice exhibited stereotypical PAH symptoms including increased hematocrit, RBC, HGB and enlarged right ventricle volume. Nelson Grihalde grihalde@otm.uic.edu 312-996-4129