The molecular etiology is covered by reviews on the transcriptional regulation of the ABCC6 gene and the role of modifier genes in PXE, while a contribution on ENPP1 and the overlapping phenotype between PXE and Generalized Arterial Calcification of Infancy (GACI) makes the transition to clinical papers on specific aspects of the PXE retinopathy and vasculopathy. Here, ABCC6 is linked to pseudoxanthoma elasticum (inherited or acquired).