Inactivating mutations in the gene encoding ATP-binding cassette (ABC) subfamily C member 6 (ABCC6) underlie the autosomal recessive disease pseudoxanthoma elasticum (PXE, OMIM #264800) [1,2,3], characterized by ectopic mineralization in the skin, eyes, and vascular system [4,5,6]. The gene discussed is ABCC6; the disease is Pseudoxanthoma elasticum.