In line with these results, immunofluorescence staining of the FD tissue and PDO for proliferation marker, KI67, fibroblast activation marker, α-SMA, and pro-fibrotic marker, transforming growth factor beta-1 (TGF-β1), further supported the presence of proliferating cells positive for KI67, activated fibroblasts positive for α-SMA, and a TGF-β1-rich pro-fibrotic microenvironment within FD lesions, all of which were recapitulated in PDOs (Figure 4B,C). This evidence concerns the gene ACTA1 and Fabry disease.