IUBMB Enzyme Nomenclature

EC 3.6.3.4

Accepted name: Cu2+-exporting ATPase

Reaction: ATP + H2O + Cu2+in = ADP + phosphate + Cu2+out

Systematic name: ATP phosphohydrolase (Cu2+-exporting)

Comments: A P-type ATPase that undergoes covalent phosphorylation during the transport cycle. This bacterial and mammalian enzyme exports Cu2+ from cells. In humans, it is involved in Menkes disease and Wilson's disease.

Links to other databases: BRENDA, EXPASY, KEGG, Metacyc, PDB, CAS registry number:

References:

1. Vulpe, C., Levinson, B., Whitney, S., Packman, S. and Gitschier, J. Isolation of a candidate gene for Menkes disease and evidence that it encodes a copper-transporting ATPase. Nat. Genet. 3 (1993) 7-13. [PMID: 8490659]

2. Petrukhin, K., Lutsenko, S., Chernov, I., Ross, B.M., Kaplan, J.H. and Gilliam, T.C. Characterization of the Wilson disease gene encoding a P-type copper-transporting ATPase: genomic organization, alternative splicing, and structure/function predictions. Hum. Mol. Genet. 3 (1994) 1647-1656. [PMID: 7833924]

3. Fagan, M.J. and Saier, M.H., Jr. P-type ATPases of eukaryotes and bacteria: sequence analyses and construction of phylogenetic trees. J. Mol. Evol. 38 (1994) 57-99. [PMID: 8151716]

[EC 3.6.3.4 created 2000]


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