Cu2+-exporting ATPase

Cu2+-exporting ATPase

Cu2+-exporting ATPase is a type of enzyme that plays a crucial role in the transport of copper ions (Cu2+) across cellular membranes. This enzyme is responsible for maintaining the balance of copper ions within cells, which is essential for various biological processes.

Structure

Cu2+-exporting ATPase belongs to the P-type ATPase family, specifically the subfamily of heavy metal transporters. It consists of several domains, including the transmembrane domain, nucleotide-binding domain, and phosphorylation domain. These domains work together to facilitate the transport of copper ions across the membrane.

Function

The primary function of Cu2+-exporting ATPase is to pump copper ions out of the cell, thereby preventing copper overload. This process is crucial for maintaining copper homeostasis and preventing toxicity. Additionally, Cu2+-exporting ATPase is involved in the delivery of copper ions to specific cellular compartments where they are required for various enzymatic reactions.

Mechanism of Action

Cu2+-exporting ATPase utilizes the energy derived from ATP hydrolysis to transport copper ions against their concentration gradient. The enzyme undergoes conformational changes upon ATP binding and hydrolysis, which allows it to bind and release copper ions on opposite sides of the membrane. This transport mechanism ensures the efficient removal of excess copper ions from the cell.

Role in Human Health

Mutations in the gene encoding Cu2+-exporting ATPase have been associated with various human diseases, including Wilson's disease and Menkes disease. In Wilson's disease, a malfunctioning Cu2+-exporting ATPase leads to the accumulation of copper in the liver and other tissues, resulting in liver damage and neurological symptoms. Menkes disease, on the other hand, is characterized by a deficiency of Cu2+-exporting ATPase, leading to copper deficiency and impaired development.

References

1. P-type ATPase 2. Copper homeostasis 3. Wilson's disease 4. Menkes disease Template:Biochemistry