Gosset Molecular Classification
Enzyme, Transporter, P-type ATPase, P1B-type P-type cation transport ATPase
Other Names
Copper-transporting ATPase 2, Wilson disease protein, WND, Copper pump 2
Disease Roles
Copper ion transporter ATPase 7B 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.
Mechanism of Action
N/A
Biological Functions
Copper transport
Copper homeostasis
Cuproenzyme biosynthesis
Biliary excretion of copper
Disease Associations
Wilson disease
Copper accumulation
Hepatic disease
Neurological disorders
Iron metabolism defects
Safety Considerations
- Potential for drug-induced copper dysregulation
- Off-target effects on other metal transporters