Recently, it was observed that in iCCA, the TGF-β-specific gene signature includes inflammatory mediators and the potent profibrotic gene Serpine1, which encodes for plasminogen activator inhibitor-1 (PAI-1) that is an important serine protease inhibitor expressed by tumor cells, endothelial cells, and CAFs [44]. This evidence concerns the gene SERPINE1 and infantile convulsions and choreoathetosis.