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Because of its presence in blood and tissue fluids, it is considered a physiologic carrier of copper, with roles in signaling and tissue maintenance that remain under active investigation

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The speciation of copper sites in the CCS chaperone for target Cu, Zn-SOD1 and in the HAH1 chaperone for the soluble cytosolic domains (Menkes protein, MNK1) of the target ATP7A (Fig