Experimentally, constitutive EC-specific activation of TGF-β signaling in transgenic mice indeed led to a strong expression of genes related to myofibroblast activation, resulting in a severe cutaneous and pulmonary fibrosis, with important perivascular ECM deposition and subendothelial thickening of small arterioles resembling the typical SSc alterations [55]. Here, TGFB1 is linked to systemic sclerosis.