Reactome | ATP7B transports cytosolic Cu2+ to Golgi lumen

ATP7B transports cytosolic Cu2+ to Golgi lumen

Stable Identifier

R-HSA-936895

Type

Reaction [transition]

Species

Homo sapiens

Compartment

Golgi membrane

Synonyms

Copper sequestration by copper-transporting ATPase 2

ReviewStatus

5/5

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ATP7B transports cytosolic Cu2+ to Golgi lumen

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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

PubMed ID	Title	Journal	Year
8298639	The Wilson disease gene is a putative copper transporting P-type ATPase similar to the Menkes gene Rommens, JM, Thomas, GR, Bull, PC, Cox, DW, Forbes, JR	Nat Genet	1993
7626145	The Wilson disease gene: spectrum of mutations and their consequences Thomas, GR, Roberts, EA, Walshe, JM, Cox, DW, Forbes, JR	Nat Genet	1995
9307043	Two forms of Wilson disease protein produced by alternative splicing are localized in distinct cellular compartments Gilliam, T, Kawarada, Y, Miura, N, Yang, XL, Sugiyama, T, Terada, K, Petrukhin, K	Biochem J	1997