Anti-ATP7A / Copper-transporting ATPase 1

Adding 0.2 ml of distilled water will yield a concentration of 500 ug/ml. The ATP7A antibody targets Copper-transporting ATPase 1, a P-type ATPase encoded by the ATP7A gene that regulates intracellular copper distribution and homeostasis. Copper-transporting ATPase 1 facilitates the delivery of copper to cuproenzymes within the trans-Golgi network and mediates copper efflux from cells when intracellular levels rise. The ATP7A antibody provides a robust tool for investigating copper metabolism, metal ion transport, and disorders of copper imbalance such as Menkes disease.Copper-transporting ATPase 1 is a multi-domain membrane protein containing ATP-binding and transmembrane copper-binding motifs that drive active ion transport using ATP hydrolysis. It localizes mainly to the trans-Golgi network under basal conditions but relocates to the plasma membrane during copper overload. The ATP7A antibody allows researchers to study this dynamic localization, revealing how cells adapt to fluctuating copper availability to maintain metal homeostasis. ATP7A activity ensures proper metallation of key enzymes such as lysyl oxidase, dopamine beta-hydroxylase, and superoxide dismutase 1.Loss-of-function mutations in ATP7A cause Menkes disease, a lethal X-linked disorder characterized by systemic copper deficiency leading to neurodegeneration, connective tissue abnormalities, and growth retardation. The ATP7A antibody is crucial for diagnostic and mechanistic research into this condition, enabling detection of protein expression and distribution defects in patient-derived fibroblasts and tissues. Reduced expression or mislocalization of Copper-transporting ATPase 1 disrupts copper delivery to secretory enzymes, impairing multiple physiological processes.In addition to Menkes disease, partial ATP7A dysfunction underlies occipital horn syndrome, a milder disorder affecting connective tissue and autonomic function. The ATP7A antibody supports comparative studies of these phenotypes and aids in identifying molecular defects in copper trafficking. Furthermore, ATP7A expression influences tumor progression and drug resistance, as copper transport impacts angiogenesis and the uptake of platinum-based chemotherapeutics.The ATP7A antibody is validated for western blotting, immunohistochemistry, and immunofluorescence, showing clear perinuclear and membrane localization depending on copper conditions. NSJ Bioreagents provides this antibody as a high-quality reagent for consistent and sensitive detection in biochemical and disease research. By enabling precise analysis of Copper-transporting ATPase 1, the ATP7A antibody supports investigations into metal ion metabolism, neurodegeneration, and therapeutic modulation of copper-dependent enzymes.