Anti-ATOX1 antibody

Supplementary info

This supplementary information is collated from multiple sources and compiled automatically.

Activity summary

ATOX1 also known as Antioxidant 1 Copper Chaperone is a small copper-binding protein with a molecular mass of approximately 7 kDa. It plays an essential mechanical role in the transport of copper ions within the cell. ATOX1 helps deliver copper to key enzymes involved in cellular processes. It is expressed in various tissue types including the liver and the brain facilitating copper homeostasis at a cellular level.

Biological function summary

ATOX1 serves as a chaperone that transports copper ions to ATPase proteins like ATP7A and ATP7B in the secretory pathway. ATOX1 works individually rather than as part of a larger protein complex handling copper metabolism effectively. By facilitating the delivery of copper to essential enzymes ATOX1 assists in maintaining critical cellular functions such as oxidative stress response and energy production.

Pathways

Copper ion transport involving ATOX1 plays an important role in the regulation of metal ion homeostasis pathways. ATOX1 interacts closely with ATP7A and ATP7B transporting copper ions necessary for redox reactions and enzyme activities in these pathways. ATOX1's role in copper regulation also aligns with antioxidant defense processes helping protect cells from damage due to reactive oxygen species.

Associated diseases and disorders

Disruption in ATOX1 function can be linked to disorders such as Wilson's disease and Menkes disease both of which involve copper metabolism impairment. Wilson's disease involves the protein ATP7B where the absence of proper copper transport leads to toxicity. Menkes disease involves ATP7A mutations leading to a shortage of copper delivery to necessary enzymes. ATOX1's activity is important for delivering copper to these associated ATPases highlighting its importance in related pathologies.