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142 related items for PubMed ID: 28295475

  • 1. Down-regulation of hepatocyte nuclear factor-4α and defective zonation in livers expressing mutant Z α1-antitrypsin.
    Piccolo P, Annunziata P, Soria LR, Attanasio S, Barbato A, Castello R, Carissimo A, Quagliata L, Terracciano LM, Brunetti-Pierri N.
    Hepatology; 2017 Jul; 66(1):124-135. PubMed ID: 28295475
    [Abstract] [Full Text] [Related]

  • 2. 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
    [Abstract] [Full Text] [Related]

  • 3. Analyses of hepatocellular proliferation in a mouse model of alpha-1-antitrypsin deficiency.
    Rudnick DA, Liao Y, An JK, Muglia LJ, Perlmutter DH, Teckman JH.
    Hepatology; 2004 Apr; 39(4):1048-55. PubMed ID: 15057909
    [Abstract] [Full Text] [Related]

  • 4. Survival Advantage of Both Human Hepatocyte Xenografts and Genome-Edited Hepatocytes for Treatment of α-1 Antitrypsin Deficiency.
    Borel F, Tang Q, Gernoux G, Greer C, Wang Z, Barzel A, Kay MA, Shultz LD, Greiner DL, Flotte TR, Brehm MA, Mueller C.
    Mol Ther; 2017 Nov 01; 25(11):2477-2489. PubMed ID: 29032169
    [Abstract] [Full Text] [Related]

  • 5. CHOP and c-JUN up-regulate the mutant Z α1-antitrypsin, exacerbating its aggregation and liver proteotoxicity.
    Attanasio S, Ferriero R, Gernoux G, De Cegli R, Carissimo A, Nusco E, Campione S, Teckman J, Mueller C, Piccolo P, Brunetti-Pierri N.
    J Biol Chem; 2020 Sep 18; 295(38):13213-13223. PubMed ID: 32723872
    [Abstract] [Full Text] [Related]

  • 6. Alpha-1-antitrypsin deficiency: from genoma to liver disease. PiZ mouse as model for the development of liver pathology in human.
    Giovannoni I, Callea F, Stefanelli M, Mariani R, Santorelli FM, Francalanci P.
    Liver Int; 2015 Jan 18; 35(1):198-206. PubMed ID: 24529185
    [Abstract] [Full Text] [Related]

  • 7. Bone marrow stem cell therapy partially ameliorates pathological consequences in livers of mice expressing mutant human α1-antitrypsin.
    Baligar P, Kochat V, Arindkar SK, Equbal Z, Mukherjee S, Patel S, Nagarajan P, Mohanty S, Teckman JH, Mukhopadhyay A.
    Hepatology; 2017 Apr 18; 65(4):1319-1335. PubMed ID: 28056498
    [Abstract] [Full Text] [Related]

  • 8. Development of an RNAi therapeutic for alpha-1-antitrypsin liver disease.
    Wooddell CI, Blomenkamp K, Peterson RM, Subbotin VM, Schwabe C, Hamilton J, Chu Q, Christianson DR, Hegge JO, Kolbe J, Hamilton HL, Branca-Afrazi MF, Given BD, Lewis DL, Gane E, Kanner SB, Teckman JH.
    JCI Insight; 2020 Jun 18; 5(12):. PubMed ID: 32379724
    [Abstract] [Full Text] [Related]

  • 9. In vivo post-transcriptional gene silencing of alpha-1 antitrypsin by adeno-associated virus vectors expressing siRNA.
    Cruz PE, Mueller C, Cossette TL, Golant A, Tang Q, Beattie SG, Brantly M, Campbell-Thompson M, Blomenkamp KS, Teckman JH, Flotte TR.
    Lab Invest; 2007 Sep 18; 87(9):893-902. PubMed ID: 17592477
    [Abstract] [Full Text] [Related]

  • 10. Bile Duct Ligation Induces ATZ Globule Clearance in a Mouse Model of α-1 Antitrypsin Deficiency.
    Khan Z, Yokota S, Ono Y, Bell AW, Oertel M, Stolz DB, Michalopoulos GK.
    Gene Expr; 2017 Feb 10; 17(2):115-127. PubMed ID: 27938510
    [Abstract] [Full Text] [Related]

  • 11. Up-regulation of miR-34b/c by JNK and FOXO3 protects from liver fibrosis.
    Piccolo P, Ferriero R, Barbato A, Attanasio S, Monti M, Perna C, Borel F, Annunziata P, Carissimo A, De Cegli R, Quagliata L, Terracciano LM, Housset C, Teckman JH, Mueller C, Brunetti-Pierri N.
    Proc Natl Acad Sci U S A; 2021 Mar 09; 118(10):. PubMed ID: 33649241
    [Abstract] [Full Text] [Related]

  • 12. Tumour suppressor p53 down-regulates the expression of the human hepatocyte nuclear factor 4alpha (HNF4alpha) gene.
    Maeda Y, Hwang-Verslues WW, Wei G, Fukazawa T, Durbin ML, Owen LB, Liu X, Sladek FM.
    Biochem J; 2006 Dec 01; 400(2):303-13. PubMed ID: 16895524
    [Abstract] [Full Text] [Related]

  • 13. Indomethacin increases liver damage in a murine model of liver injury from alpha-1-antitrypsin deficiency.
    Rudnick DA, Shikapwashya O, Blomenkamp K, Teckman JH.
    Hepatology; 2006 Oct 01; 44(4):976-82. PubMed ID: 17006946
    [Abstract] [Full Text] [Related]

  • 14. Amelioration of Alpha-1 Antitrypsin Deficiency Diseases with Genome Editing in Transgenic Mice.
    Shen S, Sanchez ME, Blomenkamp K, Corcoran EM, Marco E, Yudkoff CJ, Jiang H, Teckman JH, Bumcrot D, Albright CF.
    Hum Gene Ther; 2018 Aug 01; 29(8):861-873. PubMed ID: 29641323
    [Abstract] [Full Text] [Related]

  • 15. Oxidative stress contributes to liver damage in a murine model of alpha-1-antitrypsin deficiency.
    Marcus NY, Blomenkamp K, Ahmad M, Teckman JH.
    Exp Biol Med (Maywood); 2012 Oct 01; 237(10):1163-72. PubMed ID: 23104507
    [Abstract] [Full Text] [Related]

  • 16. The unfolded protein response to PI*Z alpha-1 antitrypsin in human hepatocellular and murine models.
    Lu Y, Wang LR, Lee J, Mohammad NS, Aranyos AM, Gould C, Khodayari N, Oshins RA, Moneypenny CG, Brantly ML.
    Hepatol Commun; 2022 Sep 01; 6(9):2354-2367. PubMed ID: 35621045
    [Abstract] [Full Text] [Related]

  • 17. Therapeutic Genome Editing With CRISPR/Cas9 in a Humanized Mouse Model Ameliorates α1-antitrypsin Deficiency Phenotype.
    Bjursell M, Porritt MJ, Ericson E, Taheri-Ghahfarokhi A, Clausen M, Magnusson L, Admyre T, Nitsch R, Mayr L, Aasehaug L, Seeliger F, Maresca M, Bohlooly-Y M, Wiseman J.
    EBioMedicine; 2018 Mar 01; 29():104-111. PubMed ID: 29500128
    [Abstract] [Full Text] [Related]

  • 18. Hepatocyte nuclear factor-4α interacts with other hepatocyte nuclear factors in regulating transthyretin gene expression.
    Wang Z, Burke PA.
    FEBS J; 2010 Oct 01; 277(19):4066-75. PubMed ID: 20735474
    [Abstract] [Full Text] [Related]

  • 19. Alpha-1 antitrypsin Z protein (PiZ) increases hepatic fibrosis in a murine model of cholestasis.
    Mencin A, Seki E, Osawa Y, Kodama Y, De Minicis S, Knowles M, Brenner DA.
    Hepatology; 2007 Nov 01; 46(5):1443-52. PubMed ID: 17668872
    [Abstract] [Full Text] [Related]

  • 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 01; 283(5):G1156-65. PubMed ID: 12381530
    [Abstract] [Full Text] [Related]

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