Reactome: A Curated Pathway Database

ATP7A transports cytosolic Cu2+ to extracellular region

Stable Identifier
R-HSA-936802
Type
Reaction
Species
Homo sapiens
Compartment
Synonyms
Copper export from cells by copper-transporting ATPase1
Locations in the PathwayBrowser
Summation

The human gene ATP7A (MNK) encodes the copper-transporting ATPase 1 (ATP7A, ATPase1, Menkes protein) which is expressed in most tissues except the liver (Vulpe et al, 1993; Chelly et al, 1993). Normally, ATP7A 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).

Literature References
Participants
Participant Of
This entity is regulated by:
Title Physical Entity Activity
copper-exporting ATPase activity of ATP7A:PDZD11 [plasma membrane] ATP7A:PDZD11 [plasma membrane] copper-exporting ATPase activity (0004008)
Orthologous Events