In this regard, ABCC6 is one of these new players, whose deficiency is responsible for pseudoxanthoma elasticum (PXE), a rare and intriguing inherited disease with unexplained female 2 third prevalence and whose phenotype is characterized by ectopic calcifications in elastin-rich tissues such as the skin, the Burch’s membrane of the retina and the arterial wall. Here, ABCC6 is linked to autosomal recessive inherited pseudoxanthoma elasticum.