Metabolic dysregulation in the Atp7b^-/- Wilson's disease mouse model.

ABSTRACT: Inactivating mutations in the copper transporter Atp7b result in Wilson's disease. The Atp7b^-/- mouse develops hallmarks of Wilson's disease. The activity of several nuclear receptors decreased in Atp7b^-/- mice, and nuclear receptors are critical for maintaining metabolic homeostasis. Therefore, we anticipated that Atp7b^-/- mice would exhibit altered progression of diet-induced obesity, fatty liver, and insulin resistance. Following 10 wk on a chow or Western-type diet (40% kcal fat), parameters of glucose and lipid homeostasis were measured. Hepatic metabolites were measured by liquid chromatography-mass spectrometry and correlated with transcriptomic data. Atp7b^-/- mice fed a chow diet presented

SUBMITTER: Wooton-Kee CR 

PROVIDER: S-EPMC6994990 | biostudies-literature | 2020 Jan

REPOSITORIES: biostudies-literature