ATP7B transports cytosolic Cu2+ to Golgi lumen

Stable Identifier
Reaction [transition]
Homo sapiens
Copper sequestration by copper-transporting ATPase 2
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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
Catalyst Activity

P-type divalent copper transporter activity of ATP7B [Golgi membrane]

Orthologous Events
Cross References
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