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Literature summary extracted from
- High yield heterologous expression of wild-type and mutant Cu+-ATPase (ATP7B, Wilson disease protein) for functional characterization of catalytic activity and serine residues undergoing copper-dependent phosphorylation (2009), J. Biol. Chem., 284, 21307-21316.
Cloned(Commentary)
| EC Number | Cloned (Comment) | Organism |
|---|---|---|
| 7.2.2.8 | expressed in COS-1 cells | Homo sapiens |
| 7.2.2.9 | expression of wild-type and mutant myc-tagged ATP7Bs in COS1 cells using an adenoviral vector | Homo sapiens |
Protein Variants
| EC Number | Protein Variants | Comment | Organism |
|---|---|---|---|
| 7.2.2.8 | C983A/C985A | the mutant is less phosphorylated than the wild type enzyme | Homo sapiens |
| 7.2.2.8 | D1027N | the mutant is less phosphorylated than the wild type enzyme | Homo sapiens |
| 7.2.2.9 | C983A/C985A | site-directed mutagenesis of CXC copper binding motif in the transmembrane copper site TM6, absence of alkali labile phosphoenzyme formation | Homo sapiens |
| 7.2.2.9 | D1027N | site-directed mutagenesis of the P domain, absence of alkali labile phosphoenzyme formation | Homo sapiens |
Inhibitors
| EC Number | Inhibitors | Comment | Organism | Structure |
|---|---|---|---|---|
| 7.2.2.9 | Bathocuproine disulfonate | a copper chelator, reduced ATP7B activity | Homo sapiens |  |
Localization
| EC Number | Localization | Comment | Organism | GeneOntology No. | Textmining |
|---|---|---|---|---|---|
| 7.2.2.8 | microsome | - |
Homo sapiens | - |
- |
| 7.2.2.9 | microsome | - |
Homo sapiens | - |
- |
Metals/Ions
| EC Number | Metals/Ions | Comment | Organism | Structure |
|---|---|---|---|---|
| 7.2.2.8 | Cu+ | the enzyme contains six copper binding sites | Homo sapiens | |
| 7.2.2.9 | Mg2+ | required | Homo sapiens | |
Molecular Weight [Da]
| EC Number | Molecular Weight [Da] | Molecular Weight Maximum [Da] | Comment | Organism |
|---|---|---|---|---|
| 7.2.2.8 | 150000 | - |
gel filtration | Homo sapiens |
Natural Substrates/ Products (Substrates)
| EC Number | Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
|---|---|---|---|---|---|---|---|
| 7.2.2.8 | ATP + H2O + Cu+[side 1] | Homo sapiens | - |
ADP + phosphate + Cu+[side 2] | - |
? | |
| 7.2.2.9 | ATP + H2O + Cu2+/in | Homo sapiens | - |
ADP + phosphate + Cu2+/out | - |
? | |
Organism
| EC Number | Organism | UniProt | Comment | Textmining |
|---|---|---|---|---|
| 7.2.2.8 | Homo sapiens | - |
- |
- |
| 7.2.2.9 | Homo sapiens | - |
- |
- |
Posttranslational Modification
| EC Number | Posttranslational Modification | Comment | Organism |
|---|---|---|---|
| 7.2.2.8 | phosphoprotein | - |
Homo sapiens |
| 7.2.2.9 | phosphoprotein | ATP7B phosphorylation with ATP occurs with diphasic kinetics and is totally copper-dependent | Homo sapiens |
Purification (Commentary)
| EC Number | Purification (Comment) | Organism |
|---|---|---|
| 7.2.2.9 | recombinant myc-tagged ATP7B is isolated from the microsomal fraction of COS-1 cell homogenates | Homo sapiens |
Specific Activity [micromol/min/mg]
| EC Number | Specific Activity Minimum [µmol/min/mg] | Specific Activity Maximum [µmol/min/mg] | Comment | Organism |
|---|---|---|---|---|
| 7.2.2.9 | 0.081 | - |
partially purifed recombinant ATP7B in microsomes, copper-dependent, steady-state ATPase, 37°C, pH 6.0, in presence of copper and copper chelator | Homo sapiens |
| 7.2.2.9 | 0.112 | - |
partially purifed recombinant ATP7B in microsomes, copper-dependent, steady-state ATPase, 37°C, pH 6.0 in presence of copper | Homo sapiens |
Substrates and Products (Substrate)
| EC Number | Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|---|
| 7.2.2.8 | ATP + H2O + Cu+[side 1] | - |
Homo sapiens | ADP + phosphate + Cu+[side 2] | - |
? | |
| 7.2.2.9 | ATP + H2O + Cu2+/in | - |
Homo sapiens | ADP + phosphate + Cu2+/out | - |
? | |
| 7.2.2.9 | ATP + H2O + Cu2+/in | ATP7B phosphorylation with ATP occurs with diphasic kinetics and is totally copper-dependent | Homo sapiens | ADP + phosphate + Cu2+/out | - |
? | |
Synonyms
| EC Number | Synonyms | Comment | Organism |
|---|---|---|---|
| 7.2.2.8 | ATP7B | - |
Homo sapiens |
| 7.2.2.8 | Wilson disease protein | - |
Homo sapiens |
| 7.2.2.9 | ATP7B | - |
Homo sapiens |
| 7.2.2.9 | Cu+-ATPase | - |
Homo sapiens |
| 7.2.2.9 | Wilson disease protein | - |
Homo sapiens |
Temperature Optimum [°C]
| EC Number | Temperature Optimum [°C] | Temperature Optimum Maximum [°C] | Comment | Organism |
|---|---|---|---|---|
| 7.2.2.9 | 37 | - |
assay at | Homo sapiens |
pH Optimum
| EC Number | pH Optimum Minimum | pH Optimum Maximum | Comment | Organism |
|---|---|---|---|---|
| 7.2.2.9 | 6 | - |
assay at | Homo sapiens |
General Information
| EC Number | General Information | Comment | Organism |
|---|---|---|---|
| 7.2.2.8 | physiological function | the enzyme is required for copper homeostasis | Homo sapiens |
| 7.2.2.9 | physiological function | ATP7B is a P-type ATPase required for copper homeostasis and related to Wilson disease of humans | Homo sapiens |
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Release 2023.1 (January 2023)
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