Perlmutter D - Search Results

1

Varki A, Wills C, Perlmutter D, Woodruff D, Gage F, Moore J, Semendeferi K, Bernirschke K, Katzman R, Doolittle R, Bullock T. Varki A, et al. Among authors: perlmutter d. Science. 1998 Oct 9;282(5387):239-40. doi: 10.1126/science.282.5387.239d. Science. 1998. PMID: 9841385 No abstract available.

2

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. Hidvegi T, et al. Science. 2010 Jul 9;329(5988):229-32. doi: 10.1126/science.1190354. Epub 2010 Jun 3. Science. 2010. PMID: 20522742

3

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. Li J, et al. PLoS One. 2014 Jan 31;9(1):e87260. doi: 10.1371/journal.pone.0087260. eCollection 2014. PLoS One. 2014. PMID: 24498058 Free PMC article.

4

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. Mukherjee A, et al. Cell Death Differ. 2019 Mar;26(3):455-469. doi: 10.1038/s41418-018-0130-7. Epub 2018 May 23. Cell Death Differ. 2019. PMID: 29795336 Free PMC article.

5

Accumulation of mutant alpha1-antitrypsin Z in the endoplasmic reticulum activates caspases-4 and -12, NFkappaB, and BAP31 but not the unfolded protein response.

Hidvegi T, Schmidt BZ, Hale P, Perlmutter DH. Hidvegi T, et al. J Biol Chem. 2005 Nov 25;280(47):39002-15. doi: 10.1074/jbc.M508652200. Epub 2005 Sep 23. J Biol Chem. 2005. PMID: 16183649 Free article.

6

Regulator of G Signaling 16 is a marker for the distinct endoplasmic reticulum stress state associated with aggregated mutant alpha1-antitrypsin Z in the classical form of alpha1-antitrypsin deficiency.

Hidvegi T, Mirnics K, Hale P, Ewing M, Beckett C, Perlmutter DH. Hidvegi T, et al. J Biol Chem. 2007 Sep 21;282(38):27769-80. doi: 10.1074/jbc.M704330200. Epub 2007 Jul 17. J Biol Chem. 2007. PMID: 17635928 Free article.

7

The role of autophagy in alpha-1-antitrypsin deficiency.

Hidvegi T, Mukherjee A, Ewing M, Kemp C, Perlmutter DH. Hidvegi T, et al. Methods Enzymol. 2011;499:33-54. doi: 10.1016/B978-0-12-386471-0.00003-1. Methods Enzymol. 2011. PMID: 21683248

8

Novel treatment strategies for liver disease due to α1-antitrypsin deficiency.

Maurice N, Perlmutter DH. Maurice N, et al. Clin Transl Sci. 2012 Jun;5(3):289-94. doi: 10.1111/j.1752-8062.2011.00363.x. Epub 2012 Jan 10. Clin Transl Sci. 2012. PMID: 22686209 Free PMC article. Review.

9

Enhancing Autophagy with Drugs or Lung-directed Gene Therapy Reverses the Pathological Effects of Respiratory Epithelial Cell Proteinopathy.

Hidvegi T, Stolz DB, Alcorn JF, Yousem SA, Wang J, Leme AS, Houghton AM, Hale P, Ewing M, Cai H, Garchar EA, Pastore N, Annunziata P, Kaminski N, Pilewski J, Shapiro SD, Pak SC, Silverman GA, Brunetti-Pierri N, Perlmutter DH. Hidvegi T, et al. Among authors: perlmutter dh. J Biol Chem. 2015 Dec 11;290(50):29742-57. doi: 10.1074/jbc.M115.691253. Epub 2015 Oct 22. J Biol Chem. 2015. PMID: 26494620 Free PMC article.

10

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. Wang Y, et al. PLoS One. 2019 Jan 23;14(1):e0209748. doi: 10.1371/journal.pone.0209748. eCollection 2019. PLoS One. 2019. PMID: 30673724 Free PMC article.

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