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The Fra-2 transgenic mouse model of systemic sclerosis


Maurer, Britta; Distler, Jörg H W; Distler, Oliver (2013). The Fra-2 transgenic mouse model of systemic sclerosis. Vascular Pharmacology, 58(3):194-201.

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.

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.

Citations

11 citations in Web of Science®
12 citations in Scopus®
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Additional indexing

Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Rheumatology Clinic and Institute of Physical Medicine
Dewey Decimal Classification:610 Medicine & health
Language:English
Date:2013
Deposited On:15 Feb 2013 07:47
Last Modified:05 Apr 2016 16:27
Publisher:Elsevier
ISSN:1537-1891
Publisher DOI:https://doi.org/10.1016/j.vph.2012.12.001
PubMed ID:23232070

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