Fig. 2
From: Bone fragility in patients affected by congenital diseases non skeletal in origin
Distribution of Cu in the body. Most endogenous Cu is lost via bile, being excreted into the gastrointestinal tract. Biliar Cu combines with small amounts of Cu from pancreatic and intestinal fluids and intestinal cells and is eliminated from the body. Whole-body Cu retention increases with increased dietary intake. Little Cu is lost in the urines. The highest Cu concentrations are in the liver, brain, kidney, and heart. ATP7A: Cu is exported from the enterocytes into the blood by Cu-ATPase (ATP7A). ATP7B: export of Cu from the liver is mediated by a copper-transporting Cu-ATPase (ATP7B). CP: portal circulation