Thus, our research contributes additional insight into the fundamental mechanisms underlying the development of SSc symptoms and the role of TGF-β signaling in manifesting skin fibrosis and, at the same time, demonstrates the promising therapeutic potential of the TGF-β1 receptor ALK5 inhibitor SB 525334 for fibrosis associated with SSc. This evidence concerns the gene TGFB1 and systemic sclerosis.