The involvement of this pathway in the molecular pathology of ATS was sustained by the increased levels of CCN2/CTGF expression reported in ATS arterial tissue [98] and by the reduced expression of DCN, which is a TGF-β signaling inhibitor PG, in in vitro-grown patients’ fibroblasts [105]. The gene discussed is TGFB1; the disease is Andersen-Tawil syndrome.