ATP7A Recombinant Rabbit mAb (S-2289-106)

ATP7A (Menkes ATPase) is a copper-transporting P-type ATPase that uses ATP hydrolysis to actively pump Cu(I) across membranes, thereby maintaining cellular copper homeostasis: under normal or low copper conditions it resides in the trans-Golgi network (TGN) and delivers copper to cuproenzymes (e.g., peptidyl-α-monooxygenase, tyrosinase, lysyl oxidase), while under high copper it traffics to the plasma membrane to efflux excess copper; the 1,500-amino-acid protein contains eight transmembrane segments forming a copper channel, an ATP-binding domain, and six N-terminal cytosolic Cu(I)-binding GMTCXXC motifs, and mutations in ATP7A cause the X-linked disorder Menkes disease, characterized by systemic copper deficiency, neurodegeneration, and early death.