Open Access
Activation of JNK pathway aggravates proteotoxicity of hepatic mutant Z alpha1-antitrypsin.
Nunzia Pastore,Sergio Attanasio,Barbara Granese,Jeffrey Teckman,Andrew A. Wilson,Andrea Ballabio,And Nicola Brunetti-Pierri +6 more
Reads0
Chats0
About:
The article was published on 2017-01-01 and is currently open access. It has received 5 citations till now. The article focuses on the topics: Proteotoxicity.read more
Citations
More filters
Journal ArticleDOI
Liver Disease in Alpha-1 Antitrypsin Deficiency: Current Approaches and Future Directions.
TL;DR: There has been significant recent progress in the development of small molecule therapies with potential both to preserve the native liver and prevent hepatotoxicity.
Journal ArticleDOI
Down-regulation of hepatocyte nuclear factor-4α and defective zonation in livers expressing mutant Z α1-antitrypsin.
Pasquale Piccolo,Patrizia Annunziata,Leandro Raul Soria,Sergio Attanasio,Anna Barbato,Raffaele Castello,Annamaria Carissimo,Luca Quagliata,Luigi Terracciano,Nicola Brunetti-Pierri +9 more
TL;DR: In this article, 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.
Journal ArticleDOI
New Therapeutic Targets for Alpha-1 Antitrypsin Deficiency
TL;DR: Augmentation therapy to prevent the progression of lung disease and a range of approaches to treat the liver disease associated with the accumulation of mutant AAT are reviewed: modifying proteostasis networks that are activated by Z AAT polymers, stimulating autophagy, small interfering RNA and small molecules to block intracellular polymerization, and stem cell technology to correct the genetic defect that underlies AATD.
Journal ArticleDOI
Alpha1-antitrypsin deficiency and c-JUN
TL;DR: It is hypothesized that patients infected by H. pylori strains expressing the oncoprotein CagA would have more abundant intracellular c-JUN and therefore higher risk of developing early cirrhosis and possibly also hepatocellular carcinoma and whether eradicating H.pylori infection might slow the progression of ATZ-related liver disease.
Journal ArticleDOI
Liver: Taking out the JuNK to treat α1-antitrypsin deficiency
S. Tamir Rashid,David A. Lomas +1 more
TL;DR: New work has provided insight into the molecular pathways that underlie the liver disease that is associated with α1-antitrypsin deficiency, and has identified a novel approach to cell-based therapy.