Copper export from cells by copper-transporting ATPase1 Authored: Jassal, B, 2010-08-24 EC Number: 3.6.3.4 Edited: Jassal, B, 2010-08-24 Pubmed10401004 Pubmed10484781 Pubmed8490646 Pubmed8490659 Pubmed9147644 Reactome Database ID Release 43936802 Reactome, http://www.reactome.org ReactomeREACT_25071 Reviewed: He, L, 2010-11-15 The human gene ATP7A (MNK) encode the copper-transporting ATPase 1 (ATPase1, Menkes protein) which is expressed in most tissues except the liver (Vulpe et al, 1993; Chelly et al, 1993). Normally, ATPase1 resides on the trans-Golgi membrane (Dierick et al, 1997). When cells are exposed to excessive copper levels, it is rapidly relocalized to the plasma membrane where it functions in copper efflux (Petris and Mercer, 1999). Defects in ATP7A are the cause of Menkes disease (MNKD), an X-linked recessive disorder of copper metabolism characterized by generalized copper deficiency (Ambrosini and Mercer, 1999).