Heather R. Lucas and Jennifer C. Lee Laboratory of Molecular Biophysics, Biochemistry and Biophysics Center National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA

Copper is an essential biological element that is vital for proper neurodevelopment and neurophysiology (Gaggelli et al., 2006; Desai and Kaler, 2008; Culotta, 2010). Many important biological functions in humans, such as cellular respiration (cytochrome c oxidase), peptide amidation (peptidylglycine α-hydroxylating monoxygenase), neurotransmitter biosynthesis (dopamine β-hydroxylase), iron metabolism (ceruloplasmin), oxidative stress release (copper-zinc superoxide dismutase), and skin pigmentation (tyrosinase), are dependent on the proper delivery of copper to specific metalloactive sites. In fact, it is estimated that a third of the genome is composed of metalloproteins (Finney and O’Halloran, 2003; Hasnain,

2004; Maret, 2010). Thus far, approximately 30 enzymes have been characterized that use copper as a cofactor (Culotta, 2010).