Lyso-Gb3 has also been implied in the AFD pathogenesis because the “in vitro” exposure of smooth muscle cells to Lyso-Gb3 promoted its proliferation [11] and the incubation of podocytes with Lyso-Gb3 induced the expression and production of extracellular matrix compounds, like fibronectin or collagen type IV [12]. Here, FN1 is linked to Nager acrofacial dysostosis.