Copper sequestration by copper-transporting ATPase 2 Authored: Jassal, B, 2010-08-24 Edited: Jassal, B, 2010-08-24 Pubmed7626145 Pubmed8298639 Pubmed9307043 Reactome Database ID Release 43936895 Reactome, http://www.reactome.org ReactomeREACT_24963 Reviewed: He, L, 2010-11-15 The human gene ATP7B encodes the copper-transporting ATPase 2 (ATPase2, Wilson's protein) which is expressed mainly in the liver, brain and kidneys (Bull et al, 1993). ATPase2 resides on the trans-Golgi membrane where it it thought to sequester copper from the cytosol into the golgi (Yang et al, 1997). Defects in ATP7B are the cause of Wilson disease (WD), an autosomal recessive disorder of copper metabolism characterized by the toxic accumulation of copper in a number of organs, particularly the liver and brain (Thomas et al, 1995).