Article
Down-regulation of hepatocyte nuclear factor-4α and defective zonation in livers expressing mutant Z α1-antitrypsin.
Hepatology (Baltimore, Md.) - 1 Jul 2017
Piccolo Pasquale, Annunziata Patrizia, Soria Leandro R, Attanasio Sergio, Barbato Anna, Castello Raffaele, Carissimo Annamaria, Quagliata Luca, Terracciano Luigi M, Brunetti-Pierri Nicola
Abstract excerpt
α1 -Antitrypsin (AAT) deficiency is one of the most common genetic disorders and the liver disease due to the Z mutant of AAT (ATZ) is a prototype of conformational disorder due to protein misfolding with consequent aberrant intermolecular protein aggregation. In the present study, we found that livers of PiZ transgenic mice expressing human ATZ have altered expression of a network of hepatocyte transcriptional...
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