Selected recent publications:

• Ding J, Yannam GR, Roy-Chowdhury N, Hidvegi T, Basma H, Rennard SI, Wong RJ, Avsar Y, Guha C, Perlmutter DH, Fox IJ, Roy-Chowdhury J.  Spontaneous hepatic repopulation in transgenic mice expressing mutant human alpha 1-antitrypsin by wild-type donor hepatocytes.  J Clin Invest 2011;121 (in press).
• Gosai SJ, Kwak JH, Luke CJ, Long OS, King DE, Kovach KJ, Johnston PA, Shun TY, Lazo JS, Perlmutter DH, Silverman GA, Pak SC.  Automated high-content live animal drug screening using C. elegans expressing the aggregation prone serpin α1-antitrypsin. 
  PLoS ONE  2010; 5:e15460; 1-16.  PMID:21103396; PMCID:PMC2980495
• Hidvegi T, Ewing M, Hale P, Dippold C, Beckett C, Kemp C, Maurice N, Mukherjee A,    Goldbach C, Watkins S, Michalopoulos G, Perlmutter DH.  An autophagy-enhancing drug promotes degradation of mutant α-antitrypsin Z and reduces hepatic fibrosis.  Science 2010; 329:229-232.  PMID:20522742
• Klionsky DJ, et al.  Guidelines for the use and interpretation of assays for monitoring
  autophagy in higher eukaryotes.  Autophagy 2008; 4:151-175.  PMID:18188003; PMCID:PMC2654259
• Scott CM, Kruse KB, Schmidt BZ, Perlmutter DH, McCracken AA, Brodsky JL.  ADD66, A gene required for the endoplasmic reticulum associated degradation (ERAD) of alpha-1-antitrypsin-Z in yeast, facilitates proteasome activity and assembly.  Mol Biol Cell 2007; 18:3776-3787.  PMID:17634286; PMCID:PMC1995736
• Hidvegi T, Mirnics K, Hale P, Ewing M, Beckett C, Perlmutter DH.  Regulator of G signaling 16 is a marker for the distinct ER stress state associated with aggregated mutant α1-antitrypsin Z in the classical form of α1-antitrypsin deficiency. J Biol Chem 2007; 282:27769-27780.  PMID:17635928
• Kamimoto T, Shoji S, Hidvegi T, Mizushima N, Umebayashi K, Perlmutter DH, Yoshimori T.  Intracellular inclusions containing mutant α1-antitrypsin Z are propagated in the absence of autophagic activity.  J Biol Chem 2006; 281:4467-4476.  PMID:16365039
• Hidvegi T, Schmidt BZ, Hale P, Perlmutter DH.  Accumulation of mutant α1ATZ in the ER activates caspases-4 and -12, NFĸB and BAP 31 but not the unfolded protein response.  J Biol Chem 2005; 280:39002-39015.  PMID:16183649
• Schmidt BZ, Perlmutter DH.  Grp 78, Grp 94 and Grp 170 interact with alpha-1-antitrypsin mutants that are retained in the endoplasmic reticulum.  Am J Physiol 2005; 289:G444-G455.   PMID:15845869
• Rudnick DA, Liou Y, An J-K, Muglia LJ, Perlmutter DH, Teckman JH.  Analyses of hepatocellular proliferation in a mouse model of alpha-1-antitrypsin deficiency.  Hepatology 2004; 39:1048-1055.  PMID:15057909
• Teckman JH, An J-K, Blomenkamp K, Schmidt B, Perlmutter DH.  Mitochondrial autophagy and injury in the liver in alpha-1-antitrypsin deficiency.  Am J Physiol 2004; 286:G851-G862.  PMID:14684378
• Teckman JH, An J-K, Loethen S, Perlmutter DH.  Fasting in alpha-1-antitrypsin deficient liver: Constitutive activation of the autophagy.  Am J Physiol 2002; 283:G1156-G1165.  PMID:12381530

CONTACT INFORMATION

Dr. David H. Perlmutter
Phone: (412) 692-8071
Email: David.Perlmutter@chp.edu