Loss of hepatocyte β-Catenin protects mice from experimental porphyria-associated liver injury.

Journal of Hepatology(2019)

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摘要
•Porphyrias are caused by defects in heme biosynthesis, which can lead to cholestasis, inflammation, and fibrosis.•The Wnt/β-catenin pathway plays a role in pathological processes in the liver, including cholestasis and biliary injury.•Inhibiting β-catenin in a mouse model of porphyria resulted in decreased liver injury.•Several key heme biosynthesis enzymes were downregulated in livers lacking β-catenin signaling.•Mice lacking β-catenin had fewer protein aggregates, resulting in improved proteasomal activity and less autophagy.
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关键词
Heme biosynthesis,Porphyrin,Ductular proliferation,Biliary fibrosis,3,5-Diethoxycarbonyl-1,4-dihydrocollidine (DDC) diet
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