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Review

Alpha 1 antitrypsin to treat lung disease in alpha 1 antitrypsin deficiency: recent developments and clinical implications

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Pages 419-432 | Published online: 31 Jan 2018

Keep up to date with the latest research on this topic with citation updates for this article.

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Orlagh O’Shea, Saidhbhe Casey, Ciaran Giblin, Aoife Stephenson, Tomás P Carroll, Noel G McElvaney & Suzanne M McDonough. (2023) Physical Activity, Exercise Capacity and Sedentary Behavior in People with Alpha-1 Antitrypsin Deficiency: A Scoping Review. International Journal of Chronic Obstructive Pulmonary Disease 18, pages 1231-1250.
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Felix J F Herth, Robert A Sandhaus, Alice M Turner, Maria Sucena, Tobias Welte & Timm Greulich. (2021) Alpha 1 Antitrypsin Therapy in Patients with Alpha 1 Antitrypsin Deficiency: Perspectives from a Registry Study and Practical Considerations for Self-Administration During the COVID-19 Pandemic. International Journal of Chronic Obstructive Pulmonary Disease 16, pages 2983-2996.
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Peter J. Barnes. (2021) Endo-phenotyping of COPD patients. Expert Review of Respiratory Medicine 15:1, pages 27-37.
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Alessandro N. Franciosi, Mansour A. Alkhunaizi, Andrew Woodsmith, Layali Aldaihani, Hamad Alkandari, Siobhán E. Lee, Laura T. Fee, Noel G. McElvaney & Tomás P. Carroll. (2021) Alpha-1 Antitrypsin Deficiency and Tobacco Smoking: Exploring Risk Factors and Smoking Cessation in a Registry Population. COPD: Journal of Chronic Obstructive Pulmonary Disease 18:1, pages 76-82.
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Priya S Chukowry, Ross Gareth Edgar & Alice M Turner. (2019) Alpha 1 antitrypsin deficiency: a rare multisystem disease, predominantly affecting the lung. Expert Opinion on Orphan Drugs 7:7-8, pages 315-326.
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Articles from other publishers (26)

J.M. Hernández-Pérez, R. Ramos-Díaz, C. Vaquerizo-Pollino & J.A. Pérez. (2023) Frequency of alleles and genotypes associated with alpha-1 antitrypsin deficiency in clinical and general populations: Revelations about underdiagnosis. Pulmonology 29:3, pages 214-220.
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Bartolome Celli, Leonardo Fabbri, Gerard CrinerFernando J. MartinezDavid ManninoClaus VogelmeierMaria Montes de OcaAlberto PapiDon D. SinMeiLan K. Han & Alvar Agusti. (2022) Definition and Nomenclature of Chronic Obstructive Pulmonary Disease: Time for Its Revision. American Journal of Respiratory and Critical Care Medicine 206:11, pages 1317-1325.
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Dimitri Papukashvili, Nino Rcheulishvili, Cong Liu, Yang Ji, Yunjiao He & Peng George Wang. (2022) Self-Amplifying RNA Approach for Protein Replacement Therapy. International Journal of Molecular Sciences 23:21, pages 12884.
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Thangavelu Sangeetha, Tajuddin Nargis Begum, Balasubramanian Balamuralikrishnan, Meyyazhagan Arun, Kannan R. R. Rengasamy, Natchiappan Senthilkumar, Shanmugam Velayuthaprabhu, Muthukrishnan Saradhadevi, Palanisamy Sampathkumar & Arumugam Vijaya Anand. (2022) Influence of SERPINA1 Gene Polymorphisms on Anemia and Chronic Obstructive Pulmonary Disease. Journal of the Renin-Angiotensin-Aldosterone System 2022, pages 1-8.
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Sebastian Ley. (2022) Röntgen-Thorax als Basis und CT zur Phänotypisierung bei COPD. Pneumo News 14:4, pages 35-41.
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Xiao Liu, Kobenan G. W. Kouassi, Rita Vanbever & Mireille Dumoulin. (2022) Impact of the PEG length and PEGylation site on the structural, thermodynamic, thermal, and proteolytic stability of mono‐PEGylated alpha‐1 antitrypsin. Protein Science 31:9.
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Alon Gimmon, Lior Sherker, Lena Kojukarov, Melodie Zaknoun, Yotam Lior, Tova Fadel, Ronen Schuster, Eli C. Lewis & Eldad Silberstein. (2022) Accelerated Wound Border Closure Using a Microemulsion Containing Non-Inhibitory Recombinant α1-Antitrypsin. International Journal of Molecular Sciences 23:13, pages 7364.
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Bruno BALBI, Federica BENINI, Luciano CORDA, Angelo CORSICO, Ilaria FERRAROTTI & Nuccia GATTA. (2022) An Italian expert consensus on the management of alpha1-antitrypsin deficiency: a comprehensive set of algorithms. Panminerva Medica 64:2.
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Xiao Liu, Kevin Vanvarenberg, Kobenan Guy Wilfried Kouassi, Sohaib Mahri & Rita Vanbever. (2022) Production and characterization of mono-PEGylated alpha-1 antitrypsin for augmentation therapy. International Journal of Pharmaceutics 612, pages 121355.
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Ewa Janosz, Miriam Hetzel, Hanna Spielmann, Srinu Tumpara, Charlotte Rossdam, Marc Schwabbauer, Doreen Kloos, Constantin von Kaisenberg, Axel Schambach, Falk F. R. Buettner, Sabina Janciauskiene, Nico Lachmann & Thomas Moritz. (2021) Pulmonary transplantation of alpha-1 antitrypsin (AAT)-transgenic macrophages provides a source of functional human AAT in vivo. Gene Therapy 28:9, pages 477-493.
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Shan Guan, Max Darmstädter, Chuanfei Xu & Joseph Rosenecker. (2021) In Vitro Investigations on Optimizing and Nebulization of IVT-mRNA Formulations for Potential Pulmonary-Based Alpha-1-Antitrypsin Deficiency Treatment. Pharmaceutics 13:8, pages 1281.
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Chang-Yu Huang, Rui-Cheng Hu, Jie Li, Bin-Bin Chen & Ai-Guo Dai. (2021) α1-Antitrypsin alleviates inflammation and oxidative stress by suppressing autophagy in asthma. Cytokine 141, pages 155454.
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Matthew J. McNulty, David Z. Silberstein, Brooks T. Kuhn, Hal S. Padgett, Somen Nandi, Karen A. McDonald & Carroll E. Cross. (2021) Alpha-1 antitrypsin deficiency and recombinant protein sources with focus on plant sources: Updates, challenges and perspectives. Free Radical Biology and Medicine 163, pages 10-30.
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Jennifer Leap, Obaid Arshad, Tariq Cheema & Marvin Balaan. (2021) Pathophysiology of COPD. Critical Care Nursing Quarterly 44:1, pages 2-8.
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Orla O’Carroll, David J. Murphy & Cormac McCarthy. 2021. Diffuse Cystic Lung Diseases. Diffuse Cystic Lung Diseases 267 281 .
Simona Viglio, Paolo Iadarola, Maura D’Amato & Jan Stolk. (2020) Methods of Purification and Application Procedures of Alpha1 Antitrypsin: A Long-Lasting History. Molecules 25:17, pages 4014.
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José Luis López-Campos, Laura Carrasco Hernandez & Candelaria Caballero Eraso. (2020) Implications of a Change of Paradigm in Alpha1 Antitrypsin Deficiency Augmentation Therapy: From Biochemical to Clinical Efficacy. Journal of Clinical Medicine 9:8, pages 2526.
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Sean D. McCarthy, Héctor E. González & Brendan D. Higgins. (2020) Future Trends in Nebulized Therapies for Pulmonary Disease. Journal of Personalized Medicine 10:2, pages 37.
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Sabina Janciauskiene, David S. DeLuca, Miriam Barrecheguren, Tobias Welte, Marc Miravitlles, Julio Ancochea, Carlos Badiola, Guadalupe Sánchez, Enric Duran, Francisco García Río, Marc Miravitlles, Luis Muñoz, Víctor Sobradillo, Joan B. Soriano, Julio Ancochea, Luis Borderias, Francisco García Río, Jaime Martínez, Teodoro Montemayor, Luis Muñoz, Luis Piñeiro, Joan Serra, Juan José Soler-Cataluña, Antoni Torres & José Luis Viejo. (2020) Serum Levels of Alpha1-antitrypsin and Their Relationship With COPD in the General Spanish Population. Archivos de Bronconeumología 56:2, pages 76-83.
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Sabina Janciauskiene, David S. DeLuca, Miriam Barrecheguren, Tobias Welte, Marc Miravitlles, Julio Ancochea, Carlos Badiola, Guadalupe Sánchez, Enric Duran, Francisco García Río, Marc Miravitlles, Luis Muñoz, Víctor Sobradillo, Joan B. Soriano, Julio Ancochea, Luis Borderias, Francisco García Río, Jaime Martínez, Teodoro Montemayor, Luis Muñoz, Luis Piñeiro, Joan Serra, Juan José Soler-Cataluña, Antoni Torres & José Luis Viejo. (2020) Serum Levels of Alpha1-antitrypsin and Their Relationship With COPD in the General Spanish Population. Archivos de Bronconeumología (English Edition) 56:2, pages 76-83.
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Y. Fukui, Y. Okumura, K. Uchiya, Y. Komori, K. Ogawa, T. Nikai & Y. Hasegawa. (2019) Biochemical and cellular activity of chemically synthesized elastase inhibitor (S-AFUEI) from Aspergillus fumigatus. Journal de Mycologie Médicale 29:4, pages 345-351.
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Hannah A. Blair. (2019) Human α1-proteinase inhibitor (Respreeza®) in α1-antitrypsin deficiency emphysema: a profile of its use in the EU. Drugs & Therapy Perspectives 35:8, pages 363-368.
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Peter J. Barnes. (2019) Inflammatory endotypes in COPD. Allergy.
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Jef Serré, Carolien Mathyssen, Tom Tanjeko Ajime, Hannelie Korf, Karen Maes, Nele Heulens, Conny Gysemans, Chantal Mathieu, Bart Vanaudenaerde, Wim Janssens & Ghislaine Gayan-Ramirez. (2019) Airway infection with Nontypeable Haemophilus influenzae is more rapidly eradicated in vitamin D deficient mice. The Journal of Steroid Biochemistry and Molecular Biology 187, pages 42-51.
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Leonie Diederichs, Christian Trautwein & Pavel Strnad. (2018) Nur 10 % der Patienten mit AATM erhalten eine korrekte Diagnose. Gastro-News 5:5, pages 44-48.
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Yotam Lior, Mariana Zaretsky, David E. Ochayon, Diana Lotysh, Boris M. Baranovski, Ronen Schuster, Ofer Guttman, Amir Aharoni & Eli C. Lewis. (2018) Point Mutation of a Non-Elastase-Binding Site in Human α1-Antitrypsin Alters Its Anti-Inflammatory Properties. Frontiers in Immunology 9.
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