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Journal Abstract Search

136 related items for PubMed ID: 38557779

  • 1. Variants in autophagy genes MTMR12 and FAM134A are putative modifiers of the hepatic phenotype in α1-antitrypsin deficiency.
    Tafaleng EN, Li J, Wang Y, Hidvegi T, Soto-Gutierrez A, Locke AE, Nicholas TJ, Wang YC, Pak S, Cho MH, Silverman EK, Silverman GA, Jin SC, Fox IJ, Perlmutter DH.
    Hepatology; 2024 Oct 01; 80(4):859-871. PubMed ID: 38557779
    [Abstract] [Full Text] [Related]

  • 2. 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 Oct 01; 17(6):1007-1024. PubMed ID: 38336172
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  • 3. 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 01; 163(1):270-284. PubMed ID: 35301011
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  • 6. α1-antitrypsin Deficiency: A Misfolded Secretory Protein Variant with Unique Effects on the Endoplasmic Reticulum.
    Perlmutter DH.
    Endoplasmic Reticulum Stress Dis; 2016 Sep 01; 3(1):63-72. PubMed ID: 28217691
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  • 8. The role of autophagy in alpha-1-antitrypsin deficiency.
    Hidvegi T, Mukherjee A, Ewing M, Kemp C, Perlmutter DH.
    Methods Enzymol; 2011 Sep 01; 499():33-54. PubMed ID: 21683248
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  • 9. 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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  • 12. NullCanada: A novel α1-antitrypsin allele with in cis variants Glu366Lys and Ile100Asn.
    Chen S, DeMarco ML, Estey MP, Kyle B, Parker ML, Agbor TA, Kawada P, Speevak M, Nelson TN, Mattman A.
    Clin Biochem; 2020 May 31; 79():23-27. PubMed ID: 32087139
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  • 13. A transgenic zebrafish model of hepatocyte function in human Z α1-antitrypsin deficiency.
    Yip E, Giousoh A, Fung C, Wilding B, Prakash MD, Williams C, Verkade H, Bryson-Richardson RJ, Bird PI.
    Biol Chem; 2019 Nov 26; 400(12):1603-1616. PubMed ID: 31091192
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  • 14. Alpha-1 antitrypsin and liver disease: mechanisms of injury and novel interventions.
    Teckman JH, Mangalat N.
    Expert Rev Gastroenterol Hepatol; 2015 Feb 26; 9(2):261-8. PubMed ID: 25066184
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  • 15. 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 26; 62(1):147-57. PubMed ID: 25690322
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  • 16. 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 Jul 26; 2014():459823. PubMed ID: 25025052
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  • 18. 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; 26(3):455-469. PubMed ID: 29795336
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  • 19. 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 26; 7(4):411-9. PubMed ID: 24719116
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  • 20. Fasting in alpha1-antitrypsin deficient liver: constitutive [correction of consultative] activation of autophagy.
    Teckman JH, An JK, Loethen S, Perlmutter DH.
    Am J Physiol Gastrointest Liver Physiol; 2002 Nov 26; 283(5):G1156-65. PubMed ID: 12381530
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