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Overview
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Ceruloplasmin (Cp) belongs to the family of multicopper oxidases, which are among the few enzymes able to bind molecular oxygen to perform its complete reduction to water. Ceruloplasminm literally meaning ‘a blue substance from plasma’, is a blue copper‐binding (6-7 atoms per molecule) glycoprotein from the a2-globulin fraction of human serum possessing oxidase activity towards aromatic diamines and catechol. The Ceruloplasmin found in the serum is mainly produced in liver, but it is also expressed in brain, lung, spleen and testis. Ceruloplasmin contains 95% of the copper in serum, is involved in the safe handling of oxygen in several metabolic pathways in vertebrates, and is associated with reproduction. Specialized copper sites have been recruited during evolution to provide long-range electron transfer reactivity and oxygen binding and activation in proteins destined to cope with oxygen reactivity in different organisms.Aceruloplasminaemia is an autosomal recessive disorder of iron metabolism characterized by the complete absence of Ceruloplasmin. Due to the essential role of Ceruloplasmin in iron metabolism, aceruloplasminaemic patients suffer from iron overload in tissues, and subsequent clinical complications including diabetes, retinal degeneration and neurodegeneration.
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Overview