Top ▲

P1B P-type ATPases: Cu+-ATPases C

Unless otherwise stated all data on this page refer to the human proteins. Gene information is provided for human (Hs), mouse (Mm) and rat (Rn).

Overview

Click here for help

« Hide

Copper-transporting ATPases convey copper ions across cell-surface and intracellular membranes. They consist of eight TM domains and associate with multiple copper chaperone proteins (e.g. ATOX1, O00244).

Transporters

853
Click here for help

ATP7A Show summary »

ATP7B Show summary »

Target Id 853
Nomenclature ATP7B
Previous and unofficial names Atp7a | ATPase | ATPase, Cu2+ transporting, beta polypeptide | ATPase, Cu++ transporting, beta polypeptide | ATPase, Cu++ transporting, beta polypeptide (Wilson disease) | copper pump 2 | Copper-transporting ATPase 2 | Hts | PINA | pineal night-specific ATPase | Wilson disease-associated protein
Genes ATP7B (Hs), Atp7b (Mm), Atp7b (Rn)
Ensembl ID ENSG00000123191 (Hs), ENSMUSG00000006567 (Mm), ENSRNOG00000012878 (Rn)
UniProtKB AC P35670 (Hs), Q64446 (Mm), Q64535 (Rn)
EC number
3.6.3.4
Comment Autosomal recessive genetic variants that decrease ATP7B function cause Wilson's disease. Symptoms arise due to excess copper accumulation and damage to organs including the liver, brain, kidneys, heart and eyes, as a direct result of inadequate ATP7B-mediated copper excretion into the biliary system.

NC-IUPHAR subcommittee and family contributors

Show »

How to cite this family page

Database page citation (select format):

Concise Guide to PHARMACOLOGY citation:

Alexander SPH, Fabbro D, Kelly E, Mathie AA, Peters JA, Veale EL, Armstrong JF, Faccenda E, Harding SD, Davies JA et al. (2023) The Concise Guide to PHARMACOLOGY 2023/24: Transporters. Br J Pharmacol. 180 Suppl 2:S374-469.