Synonyms:ABC34, ABCC6, ARA, EST349056, MLP1, MOAT E, MOATE, MRP6, PXE, PXE1 Background:The ATP-binding cassette transporter 6 (ABCC6) gene, also named as MRP6, is a cellular transmembrane protein transporter and belongs to ATP-binding cassette (ABC) family. ABCC6 is an ATP-dependent transporter contains two ATP-binding domains. It is mainly found in the liver, the proximal tubules of the kidneys and the intestines. ABCC6 is involved in a pathway of extracellular nucleotide metabolism and the regulation of tissue calcification. Mutations of ABCC6 leads to pseudoxanthoma elasticum (PXE) which is a rare autosomal recessive disorder. Mutations of ABCC6 can also lead generalized arterial calcification of infancy-2 (GACI2).