The Function of ATPase Copper Transporter ATP7B in Intestine.

ABSTRACT:

Background & aims

Wilson disease is a disorder of copper (Cu) misbalance caused by mutations in ATP7B. ATP7B is highly expressed in the liver-the major site of Cu accumulation in patients with Wilson disease. The intestine also expresses ATP7B, but little is known about the contribution of intestinal ATP7B to normal intestinal copper homeostasis or to Wilson disease manifestations. We characterized the role of ATP7B in mouse intestinal organoids and tissues.

Methods

We collected intestinal tissues from ATP7B-knockout (Atp7b^-/-) and control mice, and established 3-dimensional enteroids. Immunohistochemistry and x-ray fluorescence were used to characterize the distribution of ATP7B and Cu in tissues. Electron microscopy, histologic analyses, and immunoblotting were

SUBMITTER: Pierson H 

PROVIDER: S-EPMC5848507 | biostudies-literature | 2018 Jan

REPOSITORIES: biostudies-literature