The TβRII∆k-fib mouse model of systemic sclerosis expresses a kinase-deficient transforming growth factor β (TGFβ) type II receptor driven by a fibroblast-specific promoter-enhancer regulatory sequence subcloned from Col1a2, leads to ligand-dependent upregulation of TGFβ signalling in fibroblasts only, replicating key fibrotic and vasculopathic features of scleroderma. Here, TGFB1 is linked to systemic sclerosis.