Recent advances in animal models of systemic sclerosis

The Journal of Dermatology
Yoshihide Asano

Abstract

Systemic sclerosis (SSc) is a multisystem connective tissue disease characterized by the three cardinal pathological features, comprising aberrant immune activation, vasculopathy and tissue fibrosis, with unknown etiology. Although many inducible and genetic animal models mimicking the selected aspects of SSc have been well documented, the lack of models encompassing the full clinical manifestations hindered the development and preclinical testing of therapies against this disease. Under this situation, three new genetic animal models have recently been established, such as Fra2 transgenic mice, urokinase-type plasminogen activator receptor deficient mice and Klf5(+/-) ;Fli1(+/-) mice, all of which recapitulate the pathological cascade of SSc. The former two murine models demonstrate endothelial cell apoptosis and capillary loss followed by tissue fibrosis, whereas the immune systems show no remarkable abnormality. Klf5(+/-) ;Fli1(+/-) mice develop immune activation, vasculopathy and tissue fibrosis in this sequence, eventually resulting in the development of dermal fibrosis, interstitial lung disease and pulmonary vascular involvement resembling those of SSc. Because Krueppel-like factor (KLF)5 and Friend leukemia integration ...Continue Reading

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Citations

Jun 7, 2016·Drug Discovery Today·Lambert I J C BergersSusan Gibbs
Mar 27, 2018·Experimental Dermatology·Hideyuki Ujiie
Dec 7, 2018·Journal of the European Academy of Dermatology and Venereology : JEADV·T MiyagawaS Sato
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Apr 15, 2021·Advanced Biology·Andrea De PieriKarin Wuertz-Kozak
Jun 15, 2021·Frontiers in Cellular and Infection Microbiology·Jungen TangLingli Dong

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