154 related articles for article (PubMed ID: 38336172)
1. Multiple Genes Core to ERAD, Macroautophagy and Lysosomal Degradation Pathways Participate in the Proteostasis Response in α1-Antitrypsin Deficiency.
Li J; Moretti F; Hidvegi T; Sviben S; Fitzpatrick JAJ; Sundaramoorthi H; Pak SC; Silverman GA; Knapp B; Filipuzzi I; Alford J; Reece-Hoyes J; Nigsch F; Murphy LO; Nyfeler B; Perlmutter DH
Cell Mol Gastroenterol Hepatol; 2024; 17(6):1007-1024. PubMed ID: 38336172
[TBL] [Abstract][Full Text] [Related]
2. A C. elegans model of human α1-antitrypsin deficiency links components of the RNAi pathway to misfolded protein turnover.
Long OS; Benson JA; Kwak JH; Luke CJ; Gosai SJ; O'Reilly LP; Wang Y; Li J; Vetica AC; Miedel MT; Stolz DB; Watkins SC; Züchner S; Perlmutter DH; Silverman GA; Pak SC
Hum Mol Genet; 2014 Oct; 23(19):5109-22. PubMed ID: 24838286
[TBL] [Abstract][Full Text] [Related]
3. NFκB mitigates the pathological effects of misfolded α1-antitrypsin by activating autophagy and an integrated program of proteostasis mechanisms.
Mukherjee A; Hidvegi T; Araya P; Ewing M; Stolz DB; Perlmutter DH
Cell Death Differ; 2019 Mar; 26(3):455-469. PubMed ID: 29795336
[TBL] [Abstract][Full Text] [Related]
4. Targeting intracellular degradation pathways for treatment of liver disease caused by α1-antitrypsin deficiency.
Wang Y; Perlmutter DH
Pediatr Res; 2014 Jan; 75(1-2):133-9. PubMed ID: 24226634
[TBL] [Abstract][Full Text] [Related]
5. ER-to-lysosome-associated degradation of proteasome-resistant ATZ polymers occurs via receptor-mediated vesicular transport.
Fregno I; Fasana E; Bergmann TJ; Raimondi A; Loi M; Soldà T; Galli C; D'Antuono R; Morone D; Danieli A; Paganetti P; van Anken E; Molinari M
EMBO J; 2018 Sep; 37(17):. PubMed ID: 30076131
[TBL] [Abstract][Full Text] [Related]
6. Ubiquitin ligase SYVN1/HRD1 facilitates degradation of the SERPINA1 Z variant/α-1-antitrypsin Z variant via SQSTM1/p62-dependent selective autophagy.
Feng L; Zhang J; Zhu N; Ding Q; Zhang X; Yu J; Qiang W; Zhang Z; Ma Y; Huang D; Shen Y; Fang S; Yu Y; Wang H; Shen Y
Autophagy; 2017 Apr; 13(4):686-702. PubMed ID: 28121484
[TBL] [Abstract][Full Text] [Related]
7. The role of autophagy in alpha-1-antitrypsin deficiency: a specific cellular response in genetic diseases associated with aggregation-prone proteins.
Perlmutter DH
Autophagy; 2006; 2(4):258-63. PubMed ID: 16874089
[TBL] [Abstract][Full Text] [Related]
8. Induced pluripotent stem cells model personalized variations in liver disease resulting from α1-antitrypsin deficiency.
Tafaleng EN; Chakraborty S; Han B; Hale P; Wu W; Soto-Gutierrez A; Feghali-Bostwick CA; Wilson AA; Kotton DN; Nagaya M; Strom SC; Roy-Chowdhury J; Stolz DB; Perlmutter DH; Fox IJ
Hepatology; 2015 Jul; 62(1):147-57. PubMed ID: 25690322
[TBL] [Abstract][Full Text] [Related]
9. Intracellular inclusions containing mutant alpha1-antitrypsin Z are propagated in the absence of autophagic activity.
Kamimoto T; Shoji S; Hidvegi T; Mizushima N; Umebayashi K; Perlmutter DH; Yoshimori T
J Biol Chem; 2006 Feb; 281(7):4467-76. PubMed ID: 16365039
[TBL] [Abstract][Full Text] [Related]
10. Fluphenazine reduces proteotoxicity in C. elegans and mammalian models of alpha-1-antitrypsin deficiency.
Li J; Pak SC; O'Reilly LP; Benson JA; Wang Y; Hidvegi T; Hale P; Dippold C; Ewing M; Silverman GA; Perlmutter DH
PLoS One; 2014; 9(1):e87260. PubMed ID: 24498058
[TBL] [Abstract][Full Text] [Related]
11. Protein disulfide isomerases (PDIs) negatively regulate ebolavirus structural glycoprotein expression in the endoplasmic reticulum (ER) via the autophagy-lysosomal pathway.
Wang B; Zhang J; Liu X; Chai Q; Lu X; Yao X; Yang Z; Sun L; Johnson SF; Schwartz RC; Zheng YH
Autophagy; 2022 Oct; 18(10):2350-2367. PubMed ID: 35130104
[TBL] [Abstract][Full Text] [Related]
12. An autophagy-enhancing drug promotes degradation of mutant alpha1-antitrypsin Z and reduces hepatic fibrosis.
Hidvegi T; Ewing M; Hale P; Dippold C; Beckett C; Kemp C; Maurice N; Mukherjee A; Goldbach C; Watkins S; Michalopoulos G; Perlmutter DH
Science; 2010 Jul; 329(5988):229-32. PubMed ID: 20522742
[TBL] [Abstract][Full Text] [Related]
13. ER-to-lysosome-associated degradation acts as failsafe mechanism upon ERAD dysfunction.
Fasana E; Fregno I; Galli C; Soldà T; Molinari M
EMBO Rep; 2024 Jun; 25(6):2773-2785. PubMed ID: 38773321
[TBL] [Abstract][Full Text] [Related]
14. Regulation of PGC1α Downstream of the Insulin Signaling Pathway Plays a Role in the Hepatic Proteotoxicity of Mutant α1-Antitrypsin Deficiency Variant Z.
Rudnick DA; Huang J; Hidvegi T; Chu AS; Hale P; Munanairi A; Dietzen DJ; Cliften PF; Tycksen E; Lutkewitte AJ; Finck BN; Pak SC; Silverman GA; Perlmutter DH
Gastroenterology; 2022 Jul; 163(1):270-284. PubMed ID: 35301011
[TBL] [Abstract][Full Text] [Related]
15. α1-antitrypsin Deficiency: A Misfolded Secretory Protein Variant with Unique Effects on the Endoplasmic Reticulum.
Perlmutter DH
Endoplasmic Reticulum Stress Dis; 2016 Sep; 3(1):63-72. PubMed ID: 28217691
[TBL] [Abstract][Full Text] [Related]
16. Activating transcription factor 6 limits intracellular accumulation of mutant α(1)-antitrypsin Z and mitochondrial damage in hepatoma cells.
Smith SE; Granell S; Salcedo-Sicilia L; Baldini G; Egea G; Teckman JH; Baldini G
J Biol Chem; 2011 Dec; 286(48):41563-41577. PubMed ID: 21976666
[TBL] [Abstract][Full Text] [Related]
17. The Autophagy Pathway: A Critical Route in the Disposal of Alpha 1-Antitrypsin Aggregates That Holds Many Mysteries.
Leon C; Bouchecareilh M
Int J Mol Sci; 2021 Feb; 22(4):. PubMed ID: 33668611
[TBL] [Abstract][Full Text] [Related]
18. An analog of glibenclamide selectively enhances autophagic degradation of misfolded α1-antitrypsin Z.
Wang Y; Cobanoglu MC; Li J; Hidvegi T; Hale P; Ewing M; Chu AS; Gong Z; Muzumdar R; Pak SC; Silverman GA; Bahar I; Perlmutter DH
PLoS One; 2019; 14(1):e0209748. PubMed ID: 30673724
[TBL] [Abstract][Full Text] [Related]
19. The p24-family and COPII subunit SEC24C facilitate the clearance of alpha1-antitrypsin Z from the endoplasmic reticulum to lysosomes.
Roberts BS; Mitra D; Abishek S; Beher R; Satpute-Krishnan P
Mol Biol Cell; 2024 Mar; 35(3):ar45. PubMed ID: 38294851
[TBL] [Abstract][Full Text] [Related]
20. Capitalizing on the autophagic response for treatment of liver disease caused by alpha-1-antitrypsin deficiency and other genetic diseases.
Chu AS; Perlmutter DH; Wang Y
Biomed Res Int; 2014; 2014():459823. PubMed ID: 25025052
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]
