Additionally, recent evidence indicates that both heterozygous and compound heterozygous pathogenic variants in ENPP1, which cause Autosomal Recessive Hypophosphatemic Rickets Type 2 (ARHR2) as well as Generalized Arterial Calcification of Infancy (GACI), may also increase the risk for DISH and OPLL. The gene discussed is ENPP1; the disease is hypophosphatemic rickets, autosomal recessive, 2.