title: The Fra-2 transgenic mouse model of systemic sclerosis.
abstract: In systemic sclerosis, microvascular injury often precedes the development of fibrosis. Whereas the development of digital ulcers and skin fibrosis causes high morbidity, the affection of internal organs, in particular complications such as interstitial lung disease and pulmonary (arterial) hypertension, account for the high disease-associated mortality of these patients. Vascular animal models of systemic sclerosis are of utmost importance to study pathophysiological aspects, to identify molecular key players, and to perform interventional proof of concept-studies. So far, animal models of systemic sclerosis have mainly reflected the pro-fibrotic features of the human disease. The Fra-2 (Fos-related antigen-2) transgenic mouse model simultaneously displays both pro-fibrotic and vascular characteristics of human systemic sclerosis.
authors.affiliation: Department of Rheumatology and Center of Experimental Rheumatology, University Hospital Zurich, Gloriastrasse 25, CH-8091 Zurich, Switzerland.
authors.forename: Britta
authors.initials: B
authors.lastname: Maurer
authors.forename: Jörg H W
authors.initials: JH
authors.lastname: Distler
authors.forename: Oliver
authors.initials: O
authors.lastname: Distler
mesh_terms: D000818:Animals; D004195:Disease Models, Animal; D065627:Familial Primary Pulmonary Hypertension; D005355:Fibrosis; D051921:Fos-Related Antigen-2 / Q000235:genetics*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology / Q000503:physiopathology; D017563:Lung Diseases, Interstitial / Q000209:etiology / Q000503:physiopathology; D051379:Mice; D008822:Mice, Transgenic; D012595:Scleroderma, Systemic / Q000503:physiopathology*; D012867:Skin / Q000473:pathology
chemical_list: D051921:Fos-Related Antigen-2; C497873:Fosl2 protein, mouse