Article
Regulation of PGC1α Downstream of the Insulin Signaling Pathway Plays a Role in the Hepatic Proteotoxicity of Mutant α1-Antitrypsin Deficiency Variant Z.
Gastroenterology - 1 Jul 2022
Rudnick David A, Huang Jiansheng, Hidvegi Tunda, Chu Andrew S, Hale Pamela, Munanairi Admire, Dietzen Dennis J, Cliften Paul F, Tycksen Eric, Lutkewitte Andrew J, Finck Brian N, Pak Stephen C, Silverman Gary A, Perlmutter David H
Abstract excerpt
BACKGROUND & AIMS: Insulin signaling is known to regulate essential proteostasis mechanisms. METHODS: The analyses here examined effects of insulin signaling in the PiZ mouse model of α1-antitrypsin deficiency in which hepatocellular accumulation and proteotoxicity of the misfolded α1-antitrypsin Z variant (ATZ) causes liver fibrosis and cancer. RESULTS: We first studied the effects of breeding PiZ mice to...
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