Liver as a key organ in the supply, storage, and excretion of copper

Abstract

The liver plays an important role in the disposition of copper. Most dietary copper passes through the liver where it can be used for protein and energy production or excreted through the biliary route.

The Role of Copper as a Modifier of Lipid Metabolism

“…The liver, however, is the central organ of copper homeostasis and, as discussed above, it is greatly affected in WD. It may also be functionally affected in copper deficiency (88). Caloric excess associated with the modern Western diet is implicated in NAFLD, however caloric excess does not compensate for copper deficiency.…”

Methanobactin reverses acute liver failure in a rat model of Wilson disease

“…ATP7B is a copper-transporting ATPase that mediates copper excretion into bile (1,4), and its functional loss causes a major impairment of the ability of hepatocytes to maintain copper homeostasis at the cellular and systemic levels, resulting in persistent copper accumulation in the liver. This can lead, most likely as a result of spillover of liver copper, to deleterious effects on the brain and, in many cases, to chronic liver disease and also to fulminant hepatic failure (1,(5)(6)(7)(8).…”

“…This lifelong therapy is effective if commenced before the onset of advanced hepatic or neurologic disease (6)(7)(8). The same holds true for zinc salts, which are primarily used in mild cases of WD to decrease copper absorption via the gastrointestinal tract or as copper maintenance therapy in chelator-treated WD patients (1,5,9).…”

Anaesthetic Management of a Patient With Wilson’s Disease Posted for Emergency Lscs: A Case Report

“…ATP7B has two main intracellular roles within the liver; promoting incorporation of copper into apoceruloplasmin and excretion of excess copper into the bile. 2 Patients may present with hepatic and neurological features, extrapyramidal symptoms and Kayser-Fleischer corneal rings. Neurological manifestations include tremors, dysarthria, rigid dystonia, seizures, and psychiatric disorders.…”