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Items: 1 to 20 of 316

1.
Analysis of alpha‐1‐antitrypsin (AAT)‐regulated, glucocorticoid receptor‐dependent genes in macrophages reveals a novel host defense function of AAT
Xiyuan Bai, Junfeng Gao, Xiaoyu Guan, Drew E. Narum, Lorelenn B. Fornis, David E. Griffith, Bifeng Gao, Robert A. Sandhaus, Hua Huang, Edward D. Chan
Physiol Rep. 2024 Jul; 12(14): e16124. Published online 2024 Jul 17. doi: 10.14814/phy2.16124
PMCID:
PMC11252833
AbstractArticlePDF–4.6M
2.
Heterozygous SERPINA1 Defects and Their Impact on Clinical Manifestations of Patients with Predominantly Antibody Deficiencies
Styliani Sarrou, Ioanna Voulgaridi, Athanasia Fousika, Katerina Dadouli, Olympia Margaritopoulou, Ioannis Kakkas, Christos Hadjichristodoulou, Fani Kalala, Matthaios Speletas
Int J Mol Sci. 2024 May; 25(10): 5382. Published online 2024 May 15. doi: 10.3390/ijms25105382
PMCID:
PMC11120870
AbstractArticlePDF–246K
3.
Alpha1-antitrypsin impacts innate host–pathogen interactions with Candida albicans by stimulating fungal filamentation
Martin Jaeger, Axel Dietschmann, Sophie Austermeier, Sude Dinçer, Pauline Porschitz, Larsen Vornholz, Ralph J.A. Maas, Evelien G.G. Sprenkeler, Jürgen Ruland, Stefan Wirtz, Tania Azam, Leo A.B. Joosten, Bernhard Hube, Mihai G. Netea, Charles A. Dinarello, Mark S. Gresnigt
Virulence. 2024; 15(1): 2333367. Published online 2024 Mar 22. doi: 10.1080/21505594.2024.2333367
PMCID:
PMC11008552
AbstractArticlePDF–8.1M
4.
Neonatal cholestasis in children with Alpha-1-AT deficiency is a risk for earlier severe liver disease with male predominance
Jeffrey Teckman, Philip Rosenthal, Rosalinda V. Ignacio, Cathie Spino, Lee M. Bass, Simon Horslen, Kasper Wang, John C. Magee, Saul Karpen, Akihiro Asai, Jean P. Molleston, Robert H. Squires, Binita M. Kamath, Stephen L. Guthery, Kathleen M. Loomes, Benjamin L. Shneider, Ronald J. Sokol
Hepatol Commun. 2023 Dec; 7(12): e0345. Published online 2023 Dec 7. doi: 10.1097/HC9.0000000000000345
PMCID:
PMC10984655
AbstractArticlePDF–252K
5.
Cardiovascular disease in Alpha 1 antitrypsin deficiency: an observational study assessing the role of neutrophil proteinase activity and the suitability of validated screening tools
E. Sapey, L. E. Crowley, R. G. Edgar, D. Griffiths, S. Samanta, H. Crisford, C. E. Bolton, J. R. Hurst, R. A. Stockley
Orphanet J Rare Dis. 2024; 19: 130. Published online 2024 Mar 21. doi: 10.1186/s13023-024-03124-x
PMCID:
PMC10956254
AbstractArticlePDF–1.4M
6.
Overexpression of Alpha-1 Antitrypsin Increases the Proliferation of Mesenchymal Stem Cells by Upregulation of Cyclin D1
Bryan Wolf, Prasanth Muralidharan, Michael Y. Lee, Wei Hua, Erica Green, Hongjun Wang, Charlie Strange
Int J Mol Sci. 2024 Feb; 25(4): 2015. Published online 2024 Feb 7. doi: 10.3390/ijms25042015
PMCID:
PMC10889413
AbstractArticlePDF–4.6M
7.
Rare variants in alpha 1 antitrypsin deficiency: a systematic literature review
Ilaria Ferrarotti, Marion Wencker, Joanna Chorostowska-Wynimko
Orphanet J Rare Dis. 2024; 19: 82. Published online 2024 Feb 22. doi: 10.1186/s13023-024-03069-1
PMCID:
PMC10885523
AbstractArticlePDF–3.0M
8.
Augmentation Therapy for Severe Alpha-1 Antitrypsin Deficiency Improves Survival and Is Decoupled from Spirometric Decline—A Multinational Registry Analysis
Daniel D. Fraughen, Auyon J. Ghosh, Brian D. Hobbs, Georg-Christian Funk, Tobias Meischl, Christian F. Clarenbach, Noriane A. Sievi, Karin Schmid-Scherzer, Oliver J. McElvaney, Mark P. Murphy, Adam D. Roche, Louise Clarke, Matthew Strand, Florian Vafai-Tabrizi, Geraldine Kelly, Cedric Gunaratnam, Tomás P. Carroll, Noel G. McElvaney
Am J Respir Crit Care Med. 2023 Nov 1; 208(9): 964–974. Published online 2023 Aug 25. doi: 10.1164/rccm.202305-0863OC
PMCID:
PMC10870866
AbstractArticlePDF–1.2M
9.
Alpha-1 Antitrypsin Gene Variants in Patients without Severe Deficiency Diagnosed with Pulmonary Emphysema on Chest CT
Eduardo Laviña, Sara Lumbreras, Lara Bravo, Joan B Soriano, José Luis Izquierdo, Jose Miguel Rodríguez
Int J Chron Obstruct Pulmon Dis. 2024; 19: 353–361. Published online 2024 Feb 3. doi: 10.2147/COPD.S448593
PMCID:
PMC10849915
AbstractArticlePDF–1.7M
10.
Sirtuin3 promotes the degradation of hepatic Z alpha-1 antitrypsin through lipophagy
Brittney Poole, Regina Oshins, Zhiguang Huo, Alek Aranyos, Jesse West, Sergio Duarte, Virginia C. Clark, Thiago Beduschi, Ali Zarrinpar, Mark Brantly, Nazli Khodayari
Hepatol Commun. 2024 Feb; 8(2): e0370. Published online 2024 Jan 29. doi: 10.1097/HC9.0000000000000370
Correction in:
Hepatol Commun. 2024 Jun; 8(6): e0439.
PMCID:
PMC10830086
AbstractArticlePDF–1.4M
11.
Alpha-1-antitrypsin binds to the glucocorticoid receptor with anti-inflammatory and anti-mycobacterial significance in macrophages
Xiyuan Bai, An Bai, Michele Tomasicchio, James R. Hagman, Ashley M. Buckle, Arnav Gupta, Vineela Kadiyala, Shaun Bevers, Karina A. Serban, Kevin Kim, Zhihong Feng, Kathrin Spendier, Guy Hagen, Lorelenn Fornis, David E. Griffith, Monika Dzieciatkowska, Robert A. Sandhaus, Anthony N. Gerber, Edward D. Chan
J Immunol. Author manuscript; available in PMC 2024 Jan 31.
Published in final edited form as: J Immunol. 2022 Nov 1; 209(9): 1746–1759. Published online 2022 Sep 26. doi: 10.4049/jimmunol.2200227
PMCID:
PMC10829398
AbstractArticlePDF–1.7M
12.
Alpha-1-antitrypsin-deficiency is associated with lower cardiovascular risk: an approach based on federated learning
Daniela Zöller, Christian Haverkamp, Adeline Makoudjou, Ghislain Sofack, Saskia Kiefer, Denis Gebele, Michelle Pfaffenlehner, Martin Boeker, Harald Binder, Kapil Karki, Christian Seidemann, Bernd Schmeck, Timm Greulich, Harald Renz, Stefanie Schild, Susanne A. Seuchter, Dativa Tibyampansha, Roland Buhl, Gernot Rohde, Franziska C. Trudzinski, Robert Bals, Sabina Janciauskiene, Daiana Stolz, Sebastian Fähndrich
Respir Res. 2024; 25: 38. Published online 2024 Jan 18. doi: 10.1186/s12931-023-02607-y
PMCID:
PMC10797985
AbstractArticlePDF–1.4M
13.
Initial alpha-1 antitrypsin screening in Turkish patients with chronic obstructive pulmonary disease
Seda Tural ÖNÜR
Turk J Med Sci. 2023; 53(4): 1012–1018. Published online 2023 Jun 4. doi: 10.55730/1300-0144.5665
PMCID:
PMC10760586
AbstractArticlePDF–658K
14.
Presence of Alpha 1 Antitrypsin risk variants is not associated with histologic severity of pediatric NAFLD
Maya Zahid Khan, Corie Klepper, Sarah Orkin, Ana Catalina Arce-Clachar, Kristen Bramlage, Lin Fei, Alexander Miethke, Rohit Kohli, Stavra Xanthakos, Marialena Mouzaki
J Pediatr Gastroenterol Nutr. Author manuscript; available in PMC 2024 Aug 1.
Published in final edited form as: J Pediatr Gastroenterol Nutr. 2023 Aug 1; 77(2): 166–170. Published online 2023 May 25. doi: 10.1097/MPG.0000000000003845
PMCID:
PMC10524978
AbstractArticlePDF–74K
15.
Could the Oxidation of α1-Antitrypsin Prevent the Binding of Human Neutrophil Elastase in COVID-19 Patients?
Maura D’Amato, Monica Campagnoli, Paolo Iadarola, Paola Margherita Bignami, Marco Fumagalli, Laurent Roberto Chiarelli, Giovanni Stelitano, Federica Meloni, Pasquale Linciano, Simona Collina, Giampiero Pietrocola, Valentina Vertui, Anna Aliberti, Tommaso Fossali, Simona Viglio
Int J Mol Sci. 2023 Sep; 24(17): 13533. Published online 2023 Aug 31. doi: 10.3390/ijms241713533
PMCID:
PMC10488172
AbstractArticlePDF–3.6M
16.
The prevalence of bronchiectasis in patients with alpha-1 antitrypsin deficiency: initial report of EARCO
Robert A. Stockley, Anita Pye, Joshua De Soyza, Alice M. Turner, Marc Miravitlles, the EARCO study investigators
Orphanet J Rare Dis. 2023; 18: 243. Published online 2023 Aug 12. doi: 10.1186/s13023-023-02830-2
PMCID:
PMC10422747
AbstractArticlePDF–765K
17.
Serpin Family A Member 1 Is Prognostic and Involved in Immunological Regulation in Human Cancers
Xingwang Kuai, Jiaying Lv, Junyu Zhang, Manyu Xu, Juling Ji
Int J Mol Sci. 2023 Jul; 24(14): 11566. Published online 2023 Jul 17. doi: 10.3390/ijms241411566
PMCID:
PMC10380780
AbstractArticlePDF–5.8M
18.
Association of A1AT genetic polymorphism and NSCLC: a case- control study in Egyptian population
Aliaa N. El-Dawa, Afaf M. ElSaid, Sherif Refaat, Omali Y. El-khawaga
BMC Med Genomics. 2023; 16: 173. Published online 2023 Jul 27. doi: 10.1186/s12920-023-01608-6
PMCID:
PMC10373285
AbstractArticlePDF–1.1M
19.
Increased Prevalence of Alpha-1-Antitrypsin Deficiency in Patients with Biliary Tract Cancer and Its Associated Clinicopathological Features
Martin Cornillet, Helen Zemack, Hannes Jansson, Ernesto Sparrelid, Ewa Ellis, Niklas K. Björkström
Cells. 2023 Jun; 12(12): 1663. Published online 2023 Jun 19. doi: 10.3390/cells12121663
PMCID:
PMC10297722
AbstractArticlePDF–2.3M
20.
Distribution of alpha1 antitrypsin rare alleles in six countries: Results from the Progenika diagnostic network
José Luis Lopez-Campos, Noelia Rapun, Karen Czischke, José R. Jardim, Mariano Fernandez Acquier, Abraham Ali Munive, Hakan Günen, Estrella Drobnic, Marc Miravitlles, Lourdes Osaba
Hum Genomics. 2023; 17: 48. Published online 2023 Jun 5. doi: 10.1186/s40246-023-00497-1
PMCID:
PMC10241133
AbstractArticlePDF–3.0M

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