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1.
Regulation of PGC1α Downstream of the Insulin Signaling Pathway Plays a Role in the Hepatic Proteotoxicity of Mutant α1-Antitrypsin Deficiency Variant Z.
Gastroenterology
; 163(1): 270-284, 2022 07.
Article
in English
| MEDLINE | ID: mdl-35301011
2.
Enhancing Autophagy with Drugs or Lung-directed Gene Therapy Reverses the Pathological Effects of Respiratory Epithelial Cell Proteinopathy.
J Biol Chem
; 290(50): 29742-57, 2015 Dec 11.
Article
in English
| MEDLINE | ID: mdl-26494620
3.
A genome-wide RNAi screen identifies potential drug targets in a C. elegans model of α1-antitrypsin deficiency.
Hum Mol Genet
; 23(19): 5123-32, 2014 Oct 01.
Article
in English
| MEDLINE | ID: mdl-24838285
4.
Induced pluripotent stem cells model personalized variations in liver disease resulting from α1-antitrypsin deficiency.
Hepatology
; 62(1): 147-57, 2015 Jul.
Article
in English
| MEDLINE | ID: mdl-25690322
5.
The Arg92Cys colipase polymorphism impairs function and secretion by increasing protein misfolding.
J Lipid Res
; 54(2): 514-21, 2013 Feb.
Article
in English
| MEDLINE | ID: mdl-23204298
6.
Clathrin pit-mediated endocytosis of neutrophil elastase and cathepsin G by cancer cells.
J Biol Chem
; 287(42): 35341-35350, 2012 Oct 12.
Article
in English
| MEDLINE | ID: mdl-22915586
7.
A patient with pseudo-Addison's disease and falsely elevated thyroxine due to interference in serum cortisol and free thyroxine immunoassays by two different mechanisms.
Ann Clin Biochem
; 46(Pt 2): 172-5, 2009 Mar.
Article
in English
| MEDLINE | ID: mdl-19225029
8.
An analog of glibenclamide selectively enhances autophagic degradation of misfolded α1-antitrypsin Z.
PLoS One
; 14(1): e0209748, 2019.
Article
in English
| MEDLINE | ID: mdl-30673724
9.
Effects of hepatitis C virus infection and its recurrence after liver transplantation on functional performance and health-related quality of life.
J Gastrointest Surg
; 6(1): 108-15, 2002.
Article
in English
| MEDLINE | ID: mdl-11986026
10.
Fluphenazine reduces proteotoxicity in C. elegans and mammalian models of alpha-1-antitrypsin deficiency.
PLoS One
; 9(1): e87260, 2014.
Article
in English
| MEDLINE | ID: mdl-24498058
11.
An autophagy-enhancing drug promotes degradation of mutant alpha1-antitrypsin Z and reduces hepatic fibrosis.
Science
; 329(5988): 229-32, 2010 Jul 09.
Article
in English
| MEDLINE | ID: mdl-20522742
12.
Regulator of G Signaling 16 is a marker for the distinct endoplasmic reticulum stress state associated with aggregated mutant alpha1-antitrypsin Z in the classical form of alpha1-antitrypsin deficiency.
J Biol Chem
; 282(38): 27769-80, 2007 Sep 21.
Article
in English
| MEDLINE | ID: mdl-17635928
13.
Accumulation of mutant alpha1-antitrypsin Z in the endoplasmic reticulum activates caspases-4 and -12, NFkappaB, and BAP31 but not the unfolded protein response.
J Biol Chem
; 280(47): 39002-15, 2005 Nov 25.
Article
in English
| MEDLINE | ID: mdl-16183649
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