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Copper ion transporter ATPase 7B (ATP7B)
- Target
- ATP7B
- Molecular classification
- Enzyme, Transporter, P-type ATPase, P1B-type P-type cation transport ATPase
01
Overview
Copper ion transporter ATPase 7B (ATP7B) is a P-type ATPase responsible for the transmembrane transport of copper ions, playing a critical role in maintaining cellular and systemic copper homeostasis. It is most notably associated with Wilson disease, an autosomal recessive disorder caused by mutations in the ATP7B gene that lead to toxic copper accumulation, primarily in the liver and brain. ATP7B has two main physiological roles: exporting excess copper from hepatocytes into bile and delivering copper to cuproenzymes during their biosynthesis.
- Other names
- Copper-transporting ATPase 2Wilson disease proteinWNDCopper pump 2
02
Mechanism of action
N/A
03
Biological functions
Copper transportCopper homeostasisCuproenzyme biosynthesisBiliary excretion of copper
04
Disease associations
Wilson diseaseCopper accumulationHepatic diseaseNeurological disordersIron metabolism defects
05
Safety considerations
Potential for drug-induced copper dysregulationOff-target effects on other metal transporters
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