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Expert Opinion on Biological Therapy Volume 8, 2008 - Issue 4
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Drug Evaluations

Augmentation therapy in α-1 antitrypsin deficiency

Gustavo A Heresi Fellow Respiratory Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, Ohio 44195, USA
, MD &
James K Stoller Jean Wall Bennett Professor of Medicine, Executive Director, Leadership Development, Cleveland Clinic and Head Cleveland Clinic Lerner College of Medicine, Respiratory Institute, Cleveland Clinic Respiratory Therapy, A90, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, Ohio 44195, USA +1 216 444 1960; +1 216 445 8160; [email protected]
, MD MS
Pages 515-526 | Published online: 19 Mar 2008

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Killian Hurley, Emer P. Reeves, Tomás P. Carroll & Noel G. McElvaney. (2016) Tumor necrosis factor-α driven inflammation in alpha-1 antitrypsin deficiency: a new model of pathogenesis and treatment. Expert Review of Respiratory Medicine 10:2, pages 207-222.
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F. Kueppers. (2011) The role of augmentation therapy in alpha-1 antitrypsin deficiency. Current Medical Research and Opinion 27:3, pages 579-588.
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Ariel Modrykamien & James K Stoller. (2009) Alpha-1 antitrypsin (AAT) deficiency - what are the treatment options?. Expert Opinion on Pharmacotherapy 10:16, pages 2653-2661.
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Articles from other publishers (15)

Peter C Gøtzsche & Helle Krogh Johansen. (2016) Intravenous alpha-1 antitrypsin augmentation therapy for treating patients with alpha-1 antitrypsin deficiency and lung disease. Cochrane Database of Systematic Reviews 2016:9.
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Shweta Jha, Indraneel Sanyal & D.V. Amla. (2014) Single amino acid substitutions in recombinant plant-derived human α1-proteinase inhibitor confer enhanced stability and functional efficacy. Biochimica et Biophysica Acta (BBA) - General Subjects 1840:1, pages 416-427.
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José AlegreSandra Camprubí & Ana García-Quintana. (2013) α-1 antitrypsin and chronic fatigue syndrome: a case study from pathophysiology to clinical practice. Pain Management 3:2, pages 119-122.
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Liping Zhang, Jingni Shi, Daiming Jiang, Jacek Stupak, Jiquan Ou, Qichuan Qiu, Na An, Jianjun Li & Daichang Yang. (2013) Expression and characterization of recombinant human alpha-antitrypsin in transgenic rice seed. Journal of Biotechnology 164:2, pages 300-308.
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Shweta Jha, Saurabh Agarwal, Indraneel Sanyal, G.K. Jain & D.V. Amla. (2012) Differential subcellular targeting of recombinant human ?1-proteinase inhibitor influences yield, biological activity and in planta stability of the protein in transgenic tomato plants. Plant Science 196, pages 53-66.
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R. Siekmeier. (2010) Lung deposition of inhaled Alpha-1-proteinase inhibitor (Alpha1-PI) - problems and experience of Alpha1-PI inhalation therapy in patients with hereditary Alpha1-PI deficiency and cystic fibrosis. European Journal of Medical Research 15:S2.
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Peter C Gøtzsche & Helle Krogh Johansen. 1996. Cochrane Database of Systematic Reviews. Cochrane Database of Systematic Reviews.
Kirstin M. Heutinck, Ineke J.M. ten Berge, C. Erik Hack, Jörg Hamann & Ajda T. Rowshani. (2010) Serine proteases of the human immune system in health and disease. Molecular Immunology 47:11-12, pages 1943-1955.
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Jean-Michel Sallenave. (2010) Secretory Leukocyte Protease Inhibitor and Elafin/Trappin-2. American Journal of Respiratory Cell and Molecular Biology 42:6, pages 635-643.
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John G.T. Augoustides & Michael Andritsos. (2010) Innovations in Aortic Disease: The Ascending Aorta and Aortic Arch. Journal of Cardiothoracic and Vascular Anesthesia 24:1, pages 198-207.
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N. D. Singh & H. Daniell. 2010. The Chloroplast. The Chloroplast 263 284 .
N A Kalsheker. (2009)  1-Antitrypsin deficiency: best clinical practice. Journal of Clinical Pathology 62:10, pages 865-869.
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Mark L. BrantlyJeffrey D. ChulayLili WangChristian MuellerMargaret HumphriesL. Terry SpencerFarshid RouhaniThomas J. ConlonRoberto CalcedoMichael R. Betts, Carolyn SpencerBarry J. ByrneJames M. WilsonTerence R. Flotte. (2009) Sustained transgene expression despite T lymphocyte responses in a clinical trial of rAAV1-AAT gene therapy. Proceedings of the National Academy of Sciences 106:38, pages 16363-16368.
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M. Nadai, J. Bally, M. Vitel, C. Job, G. Tissot, J. Botterman & M. Dubald. (2008) High-level expression of active human alpha1-antitrypsin in transgenic tobacco chloroplasts. Transgenic Research 18:2, pages 173-183.
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Nicola W. Mwirigi & Charles F. Thomas. (2009) Hypersensitivity Vasculitis with Leukocytoclastic Vasculitis Associated with Alpha-1-Proteinase Inhibitor. Case Reports in Medicine 2009, pages 1-6.
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