Previous reports have shown that COMMD1 plays an important role is copper disposition but have provided little specific data. Hepatic levels of COMMD1 were increased in a normal dog with bile duct obstruction and presumed hepatic copper accumulation, but COMMD1 levels could not be judged quantitatively in liver samples from three patients with WD [20]. Likewise it
tsa trichostatin was hard to determine whether Commd1 levels were increased in a single tx-j mouse hepatic lysate [20]. Since Commd1 interacts with the Wilson ATPase’s region of copper-binding domains and the affected Bedlington terrier has an isolated defect in hepatocellular copper excretion, it seems likely that COMMD1 plays a role in biliary
excretion of copper, but exactly how remain unclear. Unlike many Cu-binding proteins, COMMD1 binds Cu(II) [28]. In contrast, in a different type of hereditary hepatic copper toxicosis found in Doberman pinschers (ATP7B and COMMD1 both normal), expression of COMMD1 was decreased significantly, as measured by real time PCR, in dogs with active hepatitis [33]; a decrease in XIAP expression was attributed to inflammation.
