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Journal Abstract Search

208 related items for PubMed ID: 28217691

  • 1. 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
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  • 3. Mysteries of α1-antitrypsin deficiency: emerging therapeutic strategies for a challenging disease.
    Ghouse R, Chu A, Wang Y, Perlmutter DH.
    Dis Model Mech; 2014 Apr; 7(4):411-9. PubMed ID: 24719116
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  • 5. 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
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  • 6. 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
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  • 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 Jul; 2(4):258-63. PubMed ID: 16874089
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  • 8. 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
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  • 9. 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 01; 23(19):5109-22. PubMed ID: 24838286
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  • 12. Calreticulin enhances the secretory trafficking of a misfolded α-1-antitrypsin.
    Mohan HM, Yang B, Dean NA, Raghavan M.
    J Biol Chem; 2020 Dec 04; 295(49):16754-16772. PubMed ID: 32978262
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  • 13. 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 Dec 04; 9(1):e87260. PubMed ID: 24498058
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  • 14. The endoplasmic reticulum degradation pathway for mutant secretory proteins alpha1-antitrypsin Z and S is distinct from that for an unassembled membrane protein.
    Teckman JH, Perlmutter DH.
    J Biol Chem; 1996 May 31; 271(22):13215-20. PubMed ID: 8662752
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  • 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 02; 286(48):41563-41577. PubMed ID: 21976666
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