Detalhe da pesquisa
1.
Variants in autophagy genes MTMR12 and FAM134A are putative modifiers of the hepatic phenotype in α1-antitrypsin deficiency.
Hepatology
; 2024 Apr 01.
Artigo
em Inglês
| MEDLINE | ID: mdl-38557779
2.
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.
Artigo
em Inglês
| MEDLINE | ID: mdl-35301011
3.
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.
Artigo
em Inglês
| MEDLINE | ID: mdl-26494620
4.
Multiple Genes Core to ERAD, Macroautophagy and Lysosomal Degradation Pathways Participate in the Proteostasis Response in α1-Antitrypsin Deficiency.
Cell Mol Gastroenterol Hepatol
; 17(6): 1007-1024, 2024.
Artigo
em Inglês
| MEDLINE | ID: mdl-38336172
5.
NFκB mitigates the pathological effects of misfolded α1-antitrypsin by activating autophagy and an integrated program of proteostasis mechanisms.
Cell Death Differ
; 26(3): 455-469, 2019 03.
Artigo
em Inglês
| MEDLINE | ID: mdl-29795336
6.
An analog of glibenclamide selectively enhances autophagic degradation of misfolded α1-antitrypsin Z.
PLoS One
; 14(1): e0209748, 2019.
Artigo
em Inglês
| MEDLINE | ID: mdl-30673724
7.
Fluphenazine reduces proteotoxicity in C. elegans and mammalian models of alpha-1-antitrypsin deficiency.
PLoS One
; 9(1): e87260, 2014.
Artigo
em Inglês
| MEDLINE | ID: mdl-24498058
8.
The role of autophagy in alpha-1-antitrypsin deficiency.
Methods Enzymol
; 499: 33-54, 2011.
Artigo
em Inglês
| MEDLINE | ID: mdl-21683248
9.
Spontaneous hepatic repopulation in transgenic mice expressing mutant human α1-antitrypsin by wild-type donor hepatocytes.
J Clin Invest
; 121(5): 1930-4, 2011 May.
Artigo
em Inglês
| MEDLINE | ID: mdl-21505264
10.
An autophagy-enhancing drug promotes degradation of mutant alpha1-antitrypsin Z and reduces hepatic fibrosis.
Science
; 329(5988): 229-32, 2010 Jul 09.
Artigo
em Inglês
| MEDLINE | ID: mdl-20522742
11.
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.
Artigo
em Inglês
| MEDLINE | ID: mdl-17635928
12.
Intracellular inclusions containing mutant alpha1-antitrypsin Z are propagated in the absence of autophagic activity.
J Biol Chem
; 281(7): 4467-76, 2006 Feb 17.
Artigo
em Inglês
| MEDLINE | ID: mdl-16365039
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.
Artigo
em Inglês
| MEDLINE | ID: mdl-16183649