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Rouhani F, Paone G, Smith NK, Krein P, Barnes P, Brantly ML. Lung neutrophil burden correlates with increased pro-inflammatory cytokines and decreased lung function in individuals with alpha(1)-antitrypsin deficiency. Chest. 2000 May;117(5 Suppl 1):250S-1S. doi: 10.1378/chest.117.5_suppl_1.250s. PubMed PMID: 10843938.
Stoller JK, Rouhani F, Brantly M, Shahin S, Dweik RA, Stocks JM, Clausen J, Campbell E, Norton F. Biochemical efficacy and safety of a new pooled human plasma alpha(1)-antitrypsin, Respitin. Chest. 2002 Jul;122(1):66-74. doi: 10.1378/chest.122.1.66. PubMed PMID: 12114340.
Cui X, Hawari F, Alsaaty S, Lawrence M, Combs CA, Geng W, Rouhani FN, Miskinis D, Levine SJ. Identification of ARTS-1 as a novel TNFR1-binding protein that promotes TNFR1 ectodomain shedding. J Clin Invest. 2002 Aug;110(4):515-26. doi: 10.1172/JCI13847. PubMed PMID: 12189246; PubMed Central PMCID: PMC150410.
Eden E, Hammel J, Rouhani FN, Brantly ML, Barker AF, Buist AS, Fallat RJ, Stoller JK, Crystal RG, Turino GM. Asthma features in severe alpha1-antitrypsin deficiency: experience of the National Heart, Lung, and Blood Institute Registry. Chest. 2003 Mar;123(3):765-71. doi: 10.1378/chest.123.3.765. PubMed PMID: 12628876.
Cui X, Rouhani FN, Hawari F, Levine SJ. An aminopeptidase, ARTS-1, is required for interleukin-6 receptor shedding. J Biol Chem. 2003 Aug 1;278(31):28677-85. doi: 10.1074/jbc.M300456200. Epub 2003 May 14. PubMed PMID: 12748171.
Cui X, Rouhani FN, Hawari F, Levine SJ. Shedding of the type II IL-1 decoy receptor requires a multifunctional aminopeptidase, aminopeptidase regulator of TNF receptor type 1 shedding. J Immunol. 2003 Dec 15;171(12):6814-9. doi: 10.4049/jimmunol.171.12.6814. PubMed PMID: 14662887.
Hawari FI, Rouhani FN, Cui X, Yu ZX, Buckley C, Kaler M, Levine SJ. Release of full-length 55-kDa TNF receptor 1 in exosome-like vesicles: a mechanism for generation of soluble cytokine receptors. Proc Natl Acad Sci U S A. 2004 Feb 3;101(5):1297-302. doi: 10.1073/pnas.0307981100. Epub 2004 Jan 26. PubMed PMID: 14745008; PubMed Central PMCID: PMC337047.
Spencer LT, Paone G, Krein PM, Rouhani FN, Rivera-Nieves J, Brantly ML. Role of human neutrophil peptides in lung inflammation associated with alpha1-antitrypsin deficiency. Am J Physiol Lung Cell Mol Physiol. 2004 Mar;286(3):L514-20. doi: 10.1152/ajplung.00099.2003. Epub 2003 Oct 31. PubMed PMID: 14594730.
Buckley CA, Rouhani FN, Kaler M, Adamik B, Hawari FI, Levine SJ. Amino-terminal TACE prodomain attenuates TNFR2 cleavage independently of the cysteine switch. Am J Physiol Lung Cell Mol Physiol. 2005 Jun;288(6):L1132-8. doi: 10.1152/ajplung.00429.2004. Epub 2005 Mar 4. PubMed PMID: 15749738.
Levine SJ, Adamik B, Hawari FI, Islam A, Yu ZX, Liao DW, Zhang J, Cui X, Rouhani FN. Proteasome inhibition induces TNFR1 shedding from human airway epithelial (NCI-H292) cells. Am J Physiol Lung Cell Mol Physiol. 2005 Aug;289(2):L233-43. doi: 10.1152/ajplung.00469.2004. Epub 2005 Apr 8. PubMed PMID: 15821012.
Rouhani FN, Meitin CA, Kaler M, Miskinis-Hilligoss D, Stylianou M, Levine SJ. Effect of tumor necrosis factor antagonism on allergen-mediated asthmatic airway inflammation. Respir Med. 2005 Sep;99(9):1175-82. doi: 10.1016/j.rmed.2005.02.031. Epub 2005 Mar 23. PubMed PMID: 16085220.
Islam A, Adamik B, Hawari FI, Ma G, Rouhani FN, Zhang J, Levine SJ. Extracellular TNFR1 release requires the calcium-dependent formation of a nucleobindin 2-ARTS-1 complex. J Biol Chem. 2006 Mar 10;281(10):6860-73. doi: 10.1074/jbc.M509397200. Epub 2006 Jan 5. PubMed PMID: 16407280.
Zhang J, Hawari FI, Shamburek RD, Adamik B, Kaler M, Islam A, Liao DW, Rouhani FN, Ingham M, Levine SJ. Circulating TNFR1 exosome-like vesicles partition with the LDL fraction of human plasma. Biochem Biophys Res Commun. 2008 Feb 8;366(2):579-84. doi: 10.1016/j.bbrc.2007.12.011. Epub 2007 Dec 17. PubMed PMID: 18078813; PubMed Central PMCID: PMC2254504.
Adamik B, Islam A, Rouhani FN, Hawari FI, Zhang J, Levine SJ. An association between RBMX, a heterogeneous nuclear ribonucleoprotein, and ARTS-1 regulates extracellular TNFR1 release. Biochem Biophys Res Commun. 2008 Jul 4;371(3):505-9. doi: 10.1016/j.bbrc.2008.04.103. Epub 2008 Apr 28. PubMed PMID: 18445477; PubMed Central PMCID: PMC2442711.
Pan S, Huang L, McPherson J, Muzny D, Rouhani F, Brantly M, Gibbs R, Sifers RN. Single nucleotide polymorphism-mediated translational suppression of endoplasmic reticulum mannosidase I modifies the onset of end-stage liver disease in alpha1-antitrypsin deficiency. Hepatology. 2009 Jul;50(1):275-81. doi: 10.1002/hep.22974. PubMed PMID: 19444872; PubMed Central PMCID: PMC2705478.
Brantly ML, Chulay JD, Wang L, Mueller C, Humphries M, Spencer LT, Rouhani F, Conlon TJ, Calcedo R, Betts MR, Spencer C, Byrne BJ, Wilson JM, Flotte TR. Sustained transgene expression despite T lymphocyte responses in a clinical trial of rAAV1-AAT gene therapy. Proc Natl Acad Sci U S A. 2009 Sep 22;106(38):16363-8. doi: 10.1073/pnas.0904514106. Epub 2009 Aug 12. PubMed PMID: 19706466; PubMed Central PMCID: PMC2752529.
Rouhani FN, Brantly ML, Markello TC, Helip-Wooley A, O'Brien K, Hess R, Huizing M, Gahl WA, Gochuico BR. Alveolar macrophage dysregulation in Hermansky-Pudlak syndrome type 1. Am J Respir Crit Care Med. 2009 Dec 1;180(11):1114-21. doi: 10.1164/rccm.200901-0023OC. Epub 2009 Sep 3. PubMed PMID: 19729668; PubMed Central PMCID: PMC2784416.
Tonelli AR, Rouhani F, Li N, Schreck P, Brantly ML. Alpha-1-antitrypsin augmentation therapy in deficient individuals enrolled in the Alpha-1 Foundation DNA and Tissue Bank. Int J Chron Obstruct Pulmon Dis. 2009;4:443-52. doi: 10.2147/copd.s8577. Epub 2009 Dec 29. PubMed PMID: 20054436; PubMed Central PMCID: PMC2802045.
McGee D, Schwarz L, McClure R, Peterka L, Rouhani F, Brantly M, Strange C. Is PiSS Alpha-1 Antitrypsin Deficiency Associated with Disease?. Pulm Med. 2010;2010:570679. doi: 10.1155/2010/570679. Epub 2010 Jun 28. PubMed PMID: 21687342; PubMed Central PMCID: PMC3099463.
Mahr AD, Edberg JC, Stone JH, Hoffman GS, St Clair EW, Specks U, Dellaripa PF, Seo P, Spiera RF, Rouhani FN, Brantly ML, Merkel PA. Alpha₁-antitrypsin deficiency-related alleles Z and S and the risk of Wegener's granulomatosis. Arthritis Rheum. 2010 Dec;62(12):3760-7. doi: 10.1002/art.27742. PubMed PMID: 20827781; PubMed Central PMCID: PMC3123032.
Flotte TR, Trapnell BC, Humphries M, Carey B, Calcedo R, Rouhani F, Campbell-Thompson M, Yachnis AT, Sandhaus RA, McElvaney NG, Mueller C, Messina LM, Wilson JM, Brantly M, Knop DR, Ye GJ, Chulay JD. Phase 2 clinical trial of a recombinant adeno-associated viral vector expressing α1-antitrypsin: interim results. Hum Gene Ther. 2011 Oct;22(10):1239-47. doi: 10.1089/hum.2011.053. Epub 2011 Aug 24. PubMed PMID: 21609134; PubMed Central PMCID: PMC3205788.
Rahaghi FF, Sandhaus RA, Brantly ML, Rouhani F, Campos MA, Strange C, Hogarth DK, Eden E, Stocks JM, Krowka MJ, Stoller JK. The prevalence of alpha-1 antitrypsin deficiency among patients found to have airflow obstruction. COPD. 2012 Aug;9(4):352-8. doi: 10.3109/15412555.2012.669433. Epub 2012 Apr 16. PubMed PMID: 22506682.
Clark VC, Dhanasekaran R, Brantly M, Rouhani F, Schreck P, Nelson DR. Liver test results do not identify liver disease in adults with α(1)-antitrypsin deficiency. Clin Gastroenterol Hepatol. 2012 Nov;10(11):1278-83. doi: 10.1016/j.cgh.2012.07.007. Epub 2012 Jul 24. PubMed PMID: 22835581; PubMed Central PMCID: PMC3482934.
Wang Y, He Y, Abraham B, Rouhani FN, Brantly ML, Scott DE, Reed JL. Cytosolic, autocrine alpha-1 proteinase inhibitor (A1PI) inhibits caspase-1 and blocks IL-1β dependent cytokine release in monocytes. PLoS One. 2012;7(11):e51078. doi: 10.1371/journal.pone.0051078. Epub 2012 Nov 30. PubMed PMID: 23226468; PubMed Central PMCID: PMC3511367.
Ye GJ, Oshins RA, Rouhani FN, Brantly ML, Chulay JD. Development, validation and use of ELISA for antibodies to human alpha-1 antitrypsin. J Immunol Methods. 2013 Feb 28;388(1-2):18-24. doi: 10.1016/j.jim.2012.11.008. Epub 2012 Nov 27. PubMed PMID: 23195820.
Ma S, Lin YY, He J, Rouhani FN, Brantly M, Turino GM. Alpha-1 antitrypsin augmentation therapy and biomarkers of elastin degradation. COPD. 2013 Aug;10(4):473-81. doi: 10.3109/15412555.2013.771163. Epub 2013 Apr 5. PubMed PMID: 23560990.
Mueller C, Chulay JD, Trapnell BC, Humphries M, Carey B, Sandhaus RA, McElvaney NG, Messina L, Tang Q, Rouhani FN, Campbell-Thompson M, Fu AD, Yachnis A, Knop DR, Ye GJ, Brantly M, Calcedo R, Somanathan S, Richman LP, Vonderheide RH, Hulme MA, Brusko TM, Wilson JM, Flotte TR. Human Treg responses allow sustained recombinant adeno-associated virus-mediated transgene expression. J Clin Invest. 2013 Dec;123(12):5310-8. doi: 10.1172/JCI70314. Epub 2013 Nov 15. PubMed PMID: 24231351; PubMed Central PMCID: PMC3859421.
Sandhaus RA, Stocks J, Rouhani FN, Brantly M, Strauss P. Biochemical efficacy and safety of a new, ready-to-use, liquid alpha-1-proteinase inhibitor, GLASSIA (alpha1-proteinase inhibitor (human), intravenous). COPD. 2014 Feb;11(1):17-25. doi: 10.3109/15412555.2013.804500. Epub 2013 Jul 3. PubMed PMID: 23822603.
Manichaikul A, Hoffman EA, Smolonska J, Gao W, Cho MH, Baumhauer H, Budoff M, Austin JH, Washko GR, Carr JJ, Kaufman JD, Pottinger T, Powell CA, Wijmenga C, Zanen P, Groen HJ, Postma DS, Wanner A, Rouhani FN, Brantly ML, Powell R, Smith BM, Rabinowitz D, Raffel LJ, Hinckley Stukovsky KD, Crapo JD, Beaty TH, Hokanson JE, Silverman EK, Dupuis J, O'Connor GT, Boezen HM, Rich SS, Barr RG. Genome-wide study of percent emphysema on computed tomography in the general population. The Multi-Ethnic Study of Atherosclerosis Lung/SNP Health Association Resource Study. Am J Respir Crit Care Med. 2014 Feb 15;189(4):408-18. doi: 10.1164/rccm.201306-1061OC. PubMed PMID: 24383474; PubMed Central PMCID: PMC3977717.
Saferali A, Lee J, Sin DD, Rouhani FN, Brantly ML, Sandford AJ. Longer telomere length in COPD patients with α1-antitrypsin deficiency independent of lung function. PLoS One. 2014;9(4):e95600. doi: 10.1371/journal.pone.0095600. eCollection 2014. PubMed PMID: 24763308; PubMed Central PMCID: PMC3998943.
Tan L, Dickens JA, Demeo DL, Miranda E, Perez J, Rashid ST, Day J, Ordoñez A, Marciniak SJ, Haq I, Barker AF, Campbell EJ, Eden E, McElvaney NG, Rennard SI, Sandhaus RA, Stocks JM, Stoller JK, Strange C, Turino G, Rouhani FN, Brantly M, Lomas DA. Circulating polymers in α1-antitrypsin deficiency. Eur Respir J. 2014 May;43(5):1501-4. doi: 10.1183/09031936.00111213. Epub 2014 Mar 6. PubMed PMID: 24603821.
Tan L, Perez J, Mela M, Miranda E, Burling KA, Rouhani FN, DeMeo DL, Haq I, Irving JA, Ordóñez A, Dickens JA, Brantly M, Marciniak SJ, Alexander GJ, Gooptu B, Lomas DA. Characterising the association of latency with α(1)-antitrypsin polymerisation using a novel monoclonal antibody. Int J Biochem Cell Biol. 2015 Jan;58:81-91. doi: 10.1016/j.biocel.2014.11.005. Epub 2014 Nov 13. PubMed PMID: 25462157; PubMed Central PMCID: PMC4305080.
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