Additionally, considering the iron-dependent post-transcriptional regulation of ALAS2, the hypothesis that iron chelation with deferiprone could decrease ALAS2 expression via IRE/IRP was tested both in vitro and in a murine model of CEP, proving its efficiency at reducing porphyrin production; porphyrin accumulation progressively decreased in red blood cells and urine, and skin photosensitivity ameliorated in CEP mice treated with deferiprone (1 or 3 mg/mL in drinking water) for 26 weeks. This evidence concerns the gene ALAS2 and Congenital erythropoietic porphyria.