This transcription factor has been identified as an important regulator of extracellular matrix production in skin fibroblasts; transgenic overexpression of Fra-2 results in increased dermal thickness and myofibroblast formation and is a mouse model for SSc (101), whereas knockdown of Fra-2 reduces both TGFβ- and PDGF-induced collagen production in primary skin fibroblasts of SSc patients (102). Here, FOSL2 is linked to systemic sclerosis.