wilsons ?

[Guest guest]

I found nothing about liver disease with a LOW COPPER level.only s that

has the high copper livels. Sorry, Joanne

Shortcut to:

http://www.clevelandclinicmeded.com/diseasemanagement/gastro/wilsons/wilsons.htm

PATHOPHYSIOLOGY

Copper is an essential cofactor for many enzymes and proteins and plays a role

in the mobilization of tissue iron stores. Copper in the diet is absorbed

relatively efficiently by the small intestine, is bound relatively loosely by

circulating plasma proteins, and is delivered to the liver from the portal

circulation. The transport of copper from the hepatocytes to bile is critical in

overall copper homeostasis since biliary excretion undergoes minimal

enterohepatic recirculation. The protein ATP7B is important in the vesicular

pathway of hepatic copper transport into bile.3 The WD gene mutation leads to

absence or diminished function of ATP7B, resulting in a decrease in biliary

copper excretion4,5 and ultimately the hepatic accumulation of copper. Along

with this failure of biliary excretion, there is also reduced incorporation of

copper into ceruloplasmin, which is a serum glycoprotein that contains six

copper atoms per molecule and is synthesized predominantly in the liver. The

process of copper incorporation into apoceruloplasmin is also dependent on

ATP7B, and this process is absent or diminished in most patients with WD,

leading to a reduced circulating level of serum ceruloplasmin in most patients.6

When copper accumulates beyond the normal safe storage capacity of the liver,

hepatocellular injury results. Furthermore, when the storage capacity of the

liver for copper is exceeded or when additional cellular copper is released

because of hepatocellular damage, levels of non-ceruloplasmin-bound copper in

the circulation are elevated and copper accumulates in multiple extrahepatic

sites, in particular the brain. As copper " spills " over to other organs from the

liver, pathologic manifestations become evident in the brain, kidneys, eyes, and

joints.

The pathologic evidence for copper accumulation in the liver evolves from early

infancy to adult life. >etc.large article