My Bibliography

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Krotova K, Khodayari N, Oshins R, Aslanidi G, Brantly ML. Neutrophil elastase promotes macrophage cell adhesion and cytokine production through the integrin-Src kinases pathway. Sci Rep. 2020 Sep 28;10(1):15874. doi: 10.1038/s41598-020-72667-3. PubMed PMID: 32981934; PubMed Central PMCID: PMC7522083.

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Khodayari N, Oshins R, Holliday LS, Clark V, Xiao Q, Marek G, Mehrad B, Brantly M. Alpha-1 antitrypsin deficient individuals have circulating extracellular vesicles with profibrogenic cargo. Cell Commun Signal. 2020 Sep 4;18(1):140. doi: 10.1186/s12964-020-00648-0. PubMed PMID: 32887613; PubMed Central PMCID: PMC7487708.

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Laffranchi M, Elliston EL, Miranda E, Perez J, Ronzoni R, Jagger AM, Heyer-Chauhan N, Brantly ML, Fra A, Lomas DA, Irving JA. Intrahepatic heteropolymerization of M and Z alpha-1-antitrypsin. JCI Insight. 2020 Jul 23;5(14). doi: 10.1172/jci.insight.135459. PubMed PMID: 32699193; PubMed Central PMCID: PMC7453904.

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Sebastian M, Hsiao CJ, Futch HS, Eisinger RS, Dumeny L, Patel S, Gobena M, Katikaneni DS, Cohen J, Carpenter AM, Spiryda L, Heldermon CD, Jin L, Brantly ML. Obesity and STING1 genotype associate with 23-valent pneumococcal vaccination efficacy. JCI Insight. 2020 May 7;5(9). doi: 10.1172/jci.insight.136141. PubMed PMID: 32376795; PubMed Central PMCID: PMC7253011.

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Brantly M, Campos M, Davis AM, D'Armiento J, Goodman K, Hanna K, O'Day M, Queenan J, Sandhaus R, Stoller J, Strange C, Teckman J, Wanner A. Detection of alpha-1 antitrypsin deficiency: the past, present and future. Orphanet J Rare Dis. 2020 Apr 19;15(1):96. doi: 10.1186/s13023-020-01352-5. Review. PubMed PMID: 32306990; PubMed Central PMCID: PMC7168939.

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Fu C, Lu Y, Williams MA, Brantly ML, Ventetuolo CE, Morel LM, Mehrad B, Scott EW, Bryant AJ. Emergency myelopoiesis contributes to immune cell exhaustion and pulmonary vascular remodelling. Br J Pharmacol. 2021 Jan;178(1):187-202. doi: 10.1111/bph.14945. Epub 2020 Feb 4. PubMed PMID: 31793661; PubMed Central PMCID: PMC8240454.

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Zhang X, Pham K, Li D, Schutte RJ, Gonzalo DH, Zhang P, Oshins R, Tan W, Brantly M, Liu C, Ostrov DA. A Novel Small Molecule Inhibits Intrahepatocellular Accumulation of Z-Variant Alpha 1-Antitrypsin In Vitro and In Vivo. Cells. 2019 Dec 6;8(12). doi: 10.3390/cells8121586. PubMed PMID: 31817705; PubMed Central PMCID: PMC6953066.

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Marek GW 3rd, Brantly M, Clark VC. Reply to: "Assessment of liver phenotype in adults with severe alpha-1 antitrypsin deficiency (Pi*ZZ genotype)". J Hepatol. 2019 Dec;71(6):1274. doi: 10.1016/j.jhep.2019.09.003. Epub 2019 Oct 9. PubMed PMID: 31606160.

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Riley L, Brantly M, Ataya A. A Woman With Dyspnea and Bronchiectasis. JAMA. 2019 Aug 13;322(6):571-572. doi: 10.1001/jama.2019.8606. PubMed PMID: 31276152.

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Oliveira AC, Fu C, Lu Y, Williams MA, Pi L, Brantly ML, Ventetuolo CE, Raizada MK, Mehrad B, Scott EW, Bryant AJ. Chemokine signaling axis between endothelial and myeloid cells regulates development of pulmonary hypertension associated with pulmonary fibrosis and hypoxia. Am J Physiol Lung Cell Mol Physiol. 2019 Oct 1;317(4):L434-L444. doi: 10.1152/ajplung.00156.2019. Epub 2019 Jul 31. PubMed PMID: 31364370; PubMed Central PMCID: PMC6842914.

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Ataya A, Riley L, Fredenburg K, Brantly M. Smoking-related diffuse cystic lung disease. Respir Med Case Rep. 2019;28:100912. doi: 10.1016/j.rmcr.2019.100912. eCollection 2019. PubMed PMID: 31384548; PubMed Central PMCID: PMC6661412.

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Zhang X, Pham K, Li D, Schutte RJ, Brantly M, Liu C, Ostrov DA. Targeting the site encoded by SERPINA1*E342K for treating alpha-1 antitrypsin deficiency-associated liver diseases. FEBS Lett. 2019 Jul;593(14):1849-1862. doi: 10.1002/1873-3468.13452. Epub 2019 Jun 6. PubMed PMID: 31116417.

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Emtiazjoo AM, Hu H, Lu L, Brantly ML. Alpha-1 Antitrypsin Attenuates Acute Lung Allograft Injury in a Rat Lung Transplant Model. Transplant Direct. 2019 Jun;5(6):e458. doi: 10.1097/TXD.0000000000000898. eCollection 2019 Jun. PubMed PMID: 31723592; PubMed Central PMCID: PMC6791593.

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Gupta N, Lee HS, Young LR, Strange C, Moss J, Singer LG, Nakata K, Barker AF, Chapman JT, Brantly ML, Stocks JM, Brown KK, Lynch JP 3rd, Goldberg HJ, Downey GP, Taveira-DaSilva AM, Krischer JP, Setchell K, Trapnell BC, Inoue Y, McCormack FX, NIH Rare Lung Disease Consortium. Analysis of the MILES cohort reveals determinants of disease progression and treatment response in lymphangioleiomyomatosis. Eur Respir J. 2019 Apr;53(4). doi: 10.1183/13993003.02066-2018. Print 2019 Apr. PubMed PMID: 30846465; PubMed Central PMCID: PMC7394189.

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Wang L, Marek GW 3rd, Hlady RA, Wagner RT, Zhao X, Clark VC, Fan AX, Liu C, Brantly M, Robertson KD. Alpha-1 Antitrypsin Deficiency Liver Disease, Mutational Homogeneity Modulated by Epigenetic Heterogeneity With Links to Obesity. Hepatology. 2019 Jul;70(1):51-66. doi: 10.1002/hep.30526. Epub 2019 Mar 20. PubMed PMID: 30681738.

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Khodayari N, Oshins R, Alli AA, Tuna KM, Holliday LS, Krotova K, Brantly M. Modulation of calreticulin expression reveals a novel exosome-mediated mechanism of Z variant α(1)-antitrypsin disposal. J Biol Chem. 2019 Apr 19;294(16):6240-6252. doi: 10.1074/jbc.RA118.006142. Epub 2019 Mar 4. PubMed PMID: 30833329; PubMed Central PMCID: PMC6484112.

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Ataya A, Riley L, Brantly ML. Serum Vascular Endothelial Growth Factor D in Cystic Lung Disease. JAMA. 2019 Jan 29;321(4):401-402. doi: 10.1001/jama.2018.20926. PubMed PMID: 30605217.

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Wildes TJ, Grippin A, Fasanya H, Dyson KA, Brantly M. Effect of atorvastatin on humoral immune response to 23-valent pneumococcal polysaccharide vaccination in healthy volunteers: The StatVax randomized clinical trial. Vaccine. 2019 Feb 28;37(10):1313-1324. doi: 10.1016/j.vaccine.2019.01.023. Epub 2019 Jan 25. PubMed PMID: 30686636; PubMed Central PMCID: PMC6379110.

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Bryant AJ, Fu C, Lu Y, Brantly ML, Mehrad B, Moldawer LL, Brusko TM, Brittain EL, West JD, Austin ED, Hamid R. A checkpoint on innate myeloid cells in pulmonary arterial hypertension. Pulm Circ. 2019 Jan-Mar;9(1):2045894018823528. doi: 10.1177/2045894018823528. Epub 2018 Dec 18. PubMed PMID: 30562157; PubMed Central PMCID: PMC6329033.

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Brantly ML, Lascano JE, Shahmohammadi A. Intravenous Alpha-1 Antitrypsin Therapy for Alpha-1 Antitrypsin Deficiency: The Current State of the Evidence. Chronic Obstr Pulm Dis. 2018 Nov 28;6(1):100-114. doi: 10.15326/jcopdf.6.1.2017.0185. Review. PubMed PMID: 30775428; PubMed Central PMCID: PMC6373587.

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Prokopenko D, Sakornsakolpat P, Fier HL, Qiao D, Parker MM, McDonald MN, Manichaikul A, Rich SS, Barr RG, Williams CJ, Brantly ML, Lange C, Beaty TH, Crapo JD, Silverman EK, Cho MH. Whole-Genome Sequencing in Severe Chronic Obstructive Pulmonary Disease. Am J Respir Cell Mol Biol. 2018 Nov;59(5):614-622. doi: 10.1165/rcmb.2018-0088OC. PubMed PMID: 29949718; PubMed Central PMCID: PMC6236690.

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Greene CM, Marciniak SJ, Teckman J, Ferrarotti I, Brantly ML, Lomas DA, Stoller JK, McElvaney NG. Author Correction: α1-Antitrypsin deficiency. Nat Rev Dis Primers. 2018 Oct 29;4(1):40. doi: 10.1038/s41572-018-0043-2. PubMed PMID: 30374103.

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Clark VC, Marek G, Liu C, Collinsworth A, Shuster J, Kurtz T, Nolte J, Brantly M. Clinical and histologic features of adults with alpha-1 antitrypsin deficiency in a non-cirrhotic cohort. J Hepatol. 2018 Dec;69(6):1357-1364. doi: 10.1016/j.jhep.2018.08.005. Epub 2018 Aug 21. PubMed PMID: 30138687.

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Ataya A, Brantly M, Riley L. Lymphangioleiomyomatosis (LAM). Am J Respir Crit Care Med. 2018 Aug 15;198(4):P7-P8. doi: 10.1164/rccm.1984P7. PubMed PMID: 30109949.

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Lee J, Arisi I, Puxeddu E, Mramba LK, Amicosante M, Swaisgood CM, Pallante M, Brantly ML, Sköld CM, Saltini C. Bronchoalveolar lavage (BAL) cells in idiopathic pulmonary fibrosis express a complex pro-inflammatory, pro-repair, angiogenic activation pattern, likely associated with macrophage iron accumulation. PLoS One. 2018;13(4):e0194803. doi: 10.1371/journal.pone.0194803. eCollection 2018. PubMed PMID: 29649237; PubMed Central PMCID: PMC5896901.

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Bryant AJ, Shenoy V, Fu C, Marek G, Lorentsen KJ, Herzog EL, Brantly ML, Avram D, Scott EW. Myeloid-derived Suppressor Cells Are Necessary for Development of Pulmonary Hypertension. Am J Respir Cell Mol Biol. 2018 Feb;58(2):170-180. doi: 10.1165/rcmb.2017-0214OC. PubMed PMID: 28862882; PubMed Central PMCID: PMC5805999.

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Barker AF, Campos MA, Brantly ML, Stocks JM, Sandhaus RA, Lee D, Steinmann K, Lin J, Sorrells S. Bioequivalence of a Liquid Formulation of Alpha(1)-Proteinase Inhibitor Compared with Prolastin®-C (Lyophilized Alpha(1)-PI) in Alpha(1)-Antitrypsin Deficiency. COPD. 2017 Dec;14(6):590-596. doi: 10.1080/15412555.2017.1376044. Epub 2017 Oct 6. PubMed PMID: 28985109.

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Leverence R, Nuttall R, Palmer R, Segal M, Wood A, Yancey F, Shuster J, Brantly M, Hromas R. Using Organizational Philosophy to Create a Self-Sustaining Compensation Plan Without Harming Academic Missions. Acad Med. 2017 Aug;92(8):1133-1137. doi: 10.1097/ACM.0000000000001484. PubMed PMID: 28746136; PubMed Central PMCID: PMC5526431.

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Foreman MG, Wilson C, DeMeo DL, Hersh CP, Beaty TH, Cho MH, Ziniti J, Curran-Everett D, Criner G, Hokanson JE, Brantly M, Rouhani FN, Sandhaus RA, Crapo JD, Silverman EK, Genetic Epidemiology of COPD (COPDGene) Investigators *. Alpha-1 Antitrypsin PiMZ Genotype Is Associated with Chronic Obstructive Pulmonary Disease in Two Racial Groups. Ann Am Thorac Soc. 2017 Aug;14(8):1280-1287. doi: 10.1513/AnnalsATS.201611-838OC. PubMed PMID: 28380308; PubMed Central PMCID: PMC5566271.

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Krotova K, Marek GW, Wang RL, Aslanidi G, Hoffman BE, Khodayari N, Rouhani FN, Brantly ML. Alpha-1 Antitrypsin-Deficient Macrophages Have Increased Matriptase-Mediated Proteolytic Activity. Am J Respir Cell Mol Biol. 2017 Aug;57(2):238-247. doi: 10.1165/rcmb.2016-0366OC. PubMed PMID: 28362108; PubMed Central PMCID: PMC5800887.

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Beiko T, Janech MG, Alekseyenko AV, Atkinson C, Coxson HO, Barth JL, Stephenson SE, Wilson CL, Schnapp LM, Barker A, Brantly M, Sandhaus RA, Silverman EK, Stoller JK, Trapnell B, Charlie S. Serum Proteins Associated with Emphysema Progression in Severe Alpha-1 Antitrypsin Deficiency. Chronic Obstr Pulm Dis. 2017 Jul 15;4(3):204-216. doi: 10.15326/jcopdf.4.3.2016.0180. PubMed PMID: 28848932; PubMed Central PMCID: PMC5556912.

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Khodayari N, Marek G, Lu Y, Krotova K, Wang RL, Brantly M. Erdj3 Has an Essential Role for Z Variant Alpha-1-Antitrypsin Degradation. J Cell Biochem. 2017 Oct;118(10):3090-3101. doi: 10.1002/jcb.26069. Epub 2017 Jun 20. PubMed PMID: 28419579; PubMed Central PMCID: PMC5575529.

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Khodayari N, Wang RL, Marek G, Krotova K, Kirst M, Liu C, Rouhani F, Brantly M. SVIP regulates Z variant alpha-1 antitrypsin retro-translocation by inhibiting ubiquitin ligase gp78. PLoS One. 2017;12(3):e0172983. doi: 10.1371/journal.pone.0172983. eCollection 2017. PubMed PMID: 28301499; PubMed Central PMCID: PMC5354272.

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Nolte JL, Ataya A, Merrill H, Childs M, Brantly M. Alpha(1)-antitrypsin Deficiency-Increased Knowledge and Diagnostic Testing after Viewing Short Instructional Video. COPD. 2017 Feb;14(1):52-55. doi: 10.1080/15412555.2016.1245280. Epub 2016 Nov 7. PubMed PMID: 27819491.

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Greene CM, Marciniak SJ, Teckman J, Ferrarotti I, Brantly ML, Lomas DA, Stoller JK, McElvaney NG. α1-Antitrypsin deficiency. Nat Rev Dis Primers. 2016 Jul 28;2:16051. doi: 10.1038/nrdp.2016.51. Review. PubMed PMID: 27465791.

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Akbar MA, Cao JJ, Lu Y, Nardo D, Chen MJ, Elshikha AS, Ahamed R, Brantly M, Holliday LS, Song S. Alpha-1 Antitrypsin Gene Therapy Ameliorates Bone Loss in Ovariectomy-Induced Osteoporosis Mouse Model. Hum Gene Ther. 2016 Sep;27(9):679-86. doi: 10.1089/hum.2016.029. Epub 2016 Jun 24. PubMed PMID: 27158796.

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Uddin MN, Sultana DA, Lorentsen KJ, Cho JJ, Kirst ME, Brantly ML, Califano D, Sant'Angelo DB, Avram D. Transcription factor Bcl11b sustains iNKT1 and iNKT2 cell programs, restricts iNKT17 cell program, and governs iNKT cell survival. Proc Natl Acad Sci U S A. 2016 Jul 5;113(27):7608-13. doi: 10.1073/pnas.1521846113. Epub 2016 Jun 21. PubMed PMID: 27330109; PubMed Central PMCID: PMC4941433.

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Sandhaus RA, Turino G, Brantly ML, Campos M, Cross CE, Goodman K, Hogarth DK, Knight SL, Stocks JM, Stoller JK, Strange C, Teckman J. The Diagnosis and Management of Alpha-1 Antitrypsin Deficiency in the Adult. Chronic Obstr Pulm Dis. 2016 Jun 6;3(3):668-682. doi: 10.15326/jcopdf.3.3.2015.0182. Review. PubMed PMID: 28848891; PubMed Central PMCID: PMC5556762.

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Chen MJ, Lu Y, Simpson NE, Beveridge MJ, Elshikha AS, Akbar MA, Tsai HY, Hinske S, Qin J, Grunwitz CR, Chen T, Brantly ML, Song S. In Situ Transplantation of Alginate Bioencapsulated Adipose Tissues Derived Stem Cells (ADSCs) via Hepatic Injection in a Mouse Model. PLoS One. 2015;10(9):e0138184. doi: 10.1371/journal.pone.0138184. eCollection 2015. PubMed PMID: 26372641; PubMed Central PMCID: PMC4570793.

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Wanner A, Groft SC, Teagarden JR, Krischer J, Davis BR, Coffey CS, Hickam DH, Teckman J, Nelson DR, McCaleb ML, Loomba R, Strange C, Sandhaus RA, Brantly M, Edelman JM, Farrugia A. Clinical Trial Design for Alpha-1 Antitrypsin Deficiency: A Model for Rare Diseases. Chronic Obstr Pulm Dis. 2015 Apr 28;2(2):177-190. doi: 10.15326/jcopdf.2.2.2015.0132. PubMed PMID: 28848840; PubMed Central PMCID: PMC5556970.

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Wegman MP, Guo MH, Bennion DM, Shankar MN, Chrzanowski SM, Goldberg LA, Xu J, Williams TA, Lu X, Hsu SI, Anton SD, Leeuwenburgh C, Brantly ML. Practicality of intermittent fasting in humans and its effect on oxidative stress and genes related to aging and metabolism. Rejuvenation Res. 2015 Apr;18(2):162-72. doi: 10.1089/rej.2014.1624. PubMed PMID: 25546413; PubMed Central PMCID: PMC4403246.

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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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Yuan Z, Mehta HJ, Mohammed K, Nasreen N, Roman R, Brantly M, Sadikot RT. TREM-1 is induced in tumor associated macrophages by cyclo-oxygenase pathway in human non-small cell lung cancer. PLoS One. 2014;9(5):e94241. doi: 10.1371/journal.pone.0094241. eCollection 2014. PubMed PMID: 24842612; PubMed Central PMCID: PMC4026315.

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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.

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Yuan Z, Syed MA, Panchal D, Joo M, Colonna M, Brantly M, Sadikot RT. Triggering receptor expressed on myeloid cells 1 (TREM-1)-mediated Bcl-2 induction prolongs macrophage survival. J Biol Chem. 2014 May 23;289(21):15118-29. doi: 10.1074/jbc.M113.536490. Epub 2014 Apr 7. PubMed PMID: 24711453; PubMed Central PMCID: PMC4031561.

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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.

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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.

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Teckman J, Pardee E, Howell RR, Mannino D, Sharp RR, Brantly M, Wanner A, Lamson J, Alpha-1 Foundation Workshop Attendees. Appropriateness of newborn screening for α1-antitrypsin deficiency. J Pediatr Gastroenterol Nutr. 2014 Feb;58(2):199-203. doi: 10.1097/MPG.0000000000000196. PubMed PMID: 24121147; PubMed Central PMCID: PMC3901803.

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Chen MJ, Lu Y, Hamazaki T, Tsai HY, Erger K, Conlon T, Elshikha AS, Li H, Srivastava A, Yao C, Brantly M, Chiodo V, Hauswirth W, Terada N, Song S. Reprogramming adipose tissue-derived mesenchymal stem cells into pluripotent stem cells by a mutant adeno-associated viral vector. Hum Gene Ther Methods. 2014 Feb;25(1):72-82. doi: 10.1089/hgtb.2013.011. Epub 2013 Dec 28. PubMed PMID: 24191859; PubMed Central PMCID: PMC3904654.

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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.