Inhibition of NAE‐dependent protein hyper‐NEDDylation in cystic cholangiocytes halts cystogenesis in experimental models of polycystic liver disease

Background Polycystic liver diseases (PLDs) are genetic inherited disorders characterized by the progressive growth of numerous intrahepatic biliary cysts, which are the main cause of morbidity. Previous studies revealed that cystic cholangiocytes are characterized by endoplasmic reticulum stress an...

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Veröffentlicht in:	United European gastroenterology journal 2021-09, Vol.9 (7), p.848-859
Hauptverfasser:	Lee‐Law, Pui Y., Olaizola, Paula, Caballero‐Camino, Francisco J., Izquierdo‐Sanchez, Laura, Rodrigues, Pedro M., Perugorria, Maria J., Azkargorta, Mikel, Elortza, Felix, Martinez‐Chantar, Maria L., Aspichueta, Patricia, Marzioni, Marco, Bujanda, Luis, Drenth, Joost P. H., Banales, Jesus M.
Format:	Artikel
Sprache:	eng
Schlagworte:	Animals Apoptosis Apoptosis - drug effects Bile Ducts - pathology Biosynthesis Cell culture Cell death Cell growth Cell Proliferation - drug effects Cell Survival - drug effects Cyclopentanes - therapeutic use Cysts Cysts - drug therapy Cysts - genetics Cysts - metabolism Cysts - pathology Endoplasmic reticulum Enzyme Inhibitors - therapeutic use Enzymes Genes hepatic cystogenesis Hepatobiliary Homeostasis Humans Kidney diseases Ligands Liver cancer Liver diseases Liver Diseases - drug therapy Liver Diseases - genetics Liver Diseases - metabolism Liver Diseases - pathology Mutation NAE NEDD8 Protein - metabolism NEDDylation Original Pathogenesis posttranslational modifications Protein Processing, Post-Translational - drug effects Protein Processing, Post-Translational - genetics Proteins Pyrimidines - therapeutic use Rats Statistical analysis Sumoylation therapy Ubiquitin-Activating Enzymes - metabolism Ubiquitin-Conjugating Enzymes - metabolism Up-Regulation
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