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.

journal_name

Vascul Pharmacol

journal_title

Vascular pharmacology

authors

Maurer B,Distler JH,Distler O

doi

10.1016/j.vph.2012.12.001

subject

Has Abstract

pub_date

2013-03-01 00:00:00

pages

194-201

issue

3

eissn

1537-1891

issn

1879-3649

pii

S1537-1891(12)00201-7

journal_volume

58

pub_type

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