Localization of the Wilson disease protein in murine intestine

Wilson disease is an inherited disorder of human copper metabolism, characterized by accumulation of copper, predominantly liver and brain. The gene defect lies in the Wilson disease protein ATP7B, a copper transporting ATPase highly active in hepatocytes. In the liver ATP7B is essential for excretion of excess copper into the bile and for copper loading of ceruloplasmin in the Golgi apparatus. The extrahepatic role of ATP7B is not yet completely understood. We analyzed the intestinal expression of ATP7B in mice by RT-PCR, Western Blot and indirect immunofluorescence. We find abundant expression of ATP7B in stom…