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Cu being a transition metal is ubiquitously engaged in biological systems to derive electrons through its participation in several enzymatic reactions. Upon bestowing the significance of Cu in biological systems, an elaborate mechanism is set forth by nature for maintaining Cu homeostasis. As a consequence, a wide variety of proteins viz., family of Cu bearing proteins, cuproenzymes, Cu transporters and Cu chaperone proteins have been manifested for enabling Cu to show its relevance in biological health. In addition, understanding the role of Cu in hepatic and neuronal functions and also in angiogenesis keeps progressing with the advent of novel molecular tools. The studies on genetic defects in Cu metabolism causing abnormalities are providing insights leading to the possible prognostic cues to alleviate the sufferings.