Pathogenic variants in the ENPP1 gene (ectonucleotide pyrophosphatase/phosphodiesterase 1; OMIM∗173335), the gene associated with Generalized Arterial Calcification of Infancy (GACI; OMIM#208000), were also found to cause PXE, although less frequently (Nitschke et al., 2012). Here, ENPP1 is linked to Generalized arterial calcification of infancy.