Reactome: A Curated Pathway Database

ATP7B transports cytosolic Cu2+ to Golgi lumen

Stable Identifier
R-HSA-936895
Type
Reaction
Species
Homo sapiens
Compartment
Synonyms
Copper sequestration by copper-transporting ATPase 2
Locations in the PathwayBrowser
Summation

The human gene ATP7B encodes the copper-transporting ATPase 2 (ATP7B, ATPase2, Wilson's protein) which is expressed mainly in the liver, brain and kidneys (Bull et al, 1993). ATP7B 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).

Literature References
Participants
Participant Of
This entity is regulated by:
Title Physical Entity Activity
copper-transporting ATPase activity of ATP7B [Golgi membrane] ATP7B [Golgi membrane] copper-transporting ATPase activity (0043682)
Orthologous Events