Evolution selected for cellular processes that would allow for increased energy production for growth but also with the ability to repair and contain toxins. Moving from anaerobic to aerobic metabolism required the cell to develop a mechanism to oxidize nutrient metals and in the process reduce oxygen to water all the while protecting against oxyradical injury. Ceruloplasmin (Cp) represents a highly conserved member of a larger protein family-the multicopper oxidases-which function as essential ferroxidases regulating the transport of iron into the iron cycle for further incorporation into red blood cells and essential iron-containing proteins. Human diseases and murine disorders associated with mutations in the Cp gene have revealed a more complicated interface between copper and iron metabolism and a complex role for metal transporters that control cell survival not only through providing the essential nutrient required for cell growth, but also by controlling redox chemistry and initiating signaling pathways that control inflammation. This review will focus on Cp, discuss the synthesis and integration of copper into the protein, review the function of Cp and the other multicopper oxidases, and highlight aceruloplasminemia-a disorder associated with tissue iron accumulation most pronounced in the central nervous system and gastrointestinal system: liver and pancreas.
|Original language||English (US)|
|Title of host publication||Clinical and Translational Perspectives on WILSON DISEASE|
|Number of pages||8|
|State||Published - Jan 1 2018|
- Iron cycle
- Multicopper oxidase
ASJC Scopus subject areas