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Items: 1 to 20 of 127

1.

α1-antitrypsin deficiency and the hepatocytes - an elegans solution to drug discovery.

O'Reilly LP, Perlmutter DH, Silverman GA, Pak SC.

Int J Biochem Cell Biol. 2014 Feb;47:109-12. doi: 10.1016/j.biocel.2013.12.006. Epub 2013 Dec 17. Review.

2.

A genome-wide RNAi screen identifies potential drug targets in a C. elegans model of α1-antitrypsin deficiency.

O'Reilly LP, Long OS, Cobanoglu MC, Benson JA, Luke CJ, Miedel MT, Hale P, Perlmutter DH, Bahar I, Silverman GA, Pak SC.

Hum Mol Genet. 2014 Oct 1;23(19):5123-32. doi: 10.1093/hmg/ddu236. Epub 2014 May 16.

3.

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. doi: 10.1038/pr.2013.190. Epub 2013 Nov 13. Review.

4.

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 Jan 31;9(1):e87260. doi: 10.1371/journal.pone.0087260. eCollection 2014.

5.

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.

PMID:
15057909
6.

Molecular pathogenesis of alpha-1-antitrypsin deficiency.

Duvoix A, Roussel BD, Lomas DA.

Rev Mal Respir. 2014 Dec;31(10):992-1002. doi: 10.1016/j.rmr.2014.03.015. Epub 2014 Nov 20. Review.

PMID:
25442121
7.
8.

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. doi: 10.1242/dmm.014092. Review.

9.

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;44(4):976-82.

PMID:
17006946
10.

Automated high-content live animal drug screening using C. elegans expressing the aggregation prone serpin α1-antitrypsin Z.

Gosai SJ, Kwak JH, Luke CJ, Long OS, King DE, Kovatch KJ, Johnston PA, Shun TY, Lazo JS, Perlmutter DH, Silverman GA, Pak SC.

PLoS One. 2010 Nov 12;5(11):e15460. doi: 10.1371/journal.pone.0015460.

11.

Hepatic progenitor cell proliferation and liver injury in α-1-antitrypsin deficiency.

Brunt EM, Blomenkamp K, Ahmed M, Ali F, Marcus N, Teckman J.

J Pediatr Gastroenterol Nutr. 2010 Nov;51(5):626-30. doi: 10.1097/MPG.0b013e3181e7ff55.

12.

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 1;23(19):5109-22. doi: 10.1093/hmg/ddu235. Epub 2014 May 16.

13.

The selective advantage of alpha1-antitrypsin deficiency.

Lomas DA.

Am J Respir Crit Care Med. 2006 May 15;173(10):1072-7. Epub 2006 Jan 26. Review.

PMID:
16439713
14.

Autophagic disposal of the aggregation-prone protein that causes liver inflammation and carcinogenesis in alpha-1-antitrypsin deficiency.

Perlmutter DH.

Cell Death Differ. 2009 Jan;16(1):39-45. doi: 10.1038/cdd.2008.103. Epub 2008 Jul 11. Review.

15.

Therapeutic targeting of misfolding and conformational change in α1-antitrypsin deficiency.

Nyon MP, Gooptu B.

Future Med Chem. 2014 Jun;6(9):1047-65. doi: 10.4155/fmc.14.58. Review.

PMID:
25068987
16.

Alpha-1 antitrypsin and liver disease: mechanisms of injury and novel interventions.

Teckman JH, Mangalat N.

Expert Rev Gastroenterol Hepatol. 2015 Feb;9(2):261-8. doi: 10.1586/17474124.2014.943187. Epub 2014 Jul 28. Review.

PMID:
25066184
17.

Deficient and Null Variants of SERPINA1 Are Proteotoxic in a Caenorhabditis elegans Model of α1-Antitrypsin Deficiency.

Cummings EE, O'Reilly LP, King DE, Silverman RM, Miedel MT, Luke CJ, Perlmutter DH, Silverman GA, Pak SC.

PLoS One. 2015 Oct 29;10(10):e0141542. doi: 10.1371/journal.pone.0141542. eCollection 2015.

18.

Alpha1-antitrypsin deficiency. 4: Molecular pathophysiology.

Lomas DA, Parfrey H.

Thorax. 2004 Jun;59(6):529-35. Review.

19.

Aberrant disulphide bonding contributes to the ER retention of alpha1-antitrypsin deficiency variants.

Ronzoni R, Berardelli R, Medicina D, Sitia R, Gooptu B, Fra AM.

Hum Mol Genet. 2016 Feb 15;25(4):642-50. doi: 10.1093/hmg/ddv501. Epub 2015 Dec 8.

PMID:
26647313
20.

Update on alpha-1 antitrypsin deficiency: New therapies.

Lomas DA, Hurst JR, Gooptu B.

J Hepatol. 2016 Aug;65(2):413-24. doi: 10.1016/j.jhep.2016.03.010. Epub 2016 Mar 29. Review.

PMID:
27034252

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