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Select item 113515701.

Prevalence of Alpha-1 Antitrypsin Deficiency Alleles in a Lithuanian Cohort of Wheezing Small Children

Edita Poluzioroviene, Joanna Chorostowska-Wynimko, Sigita Petraitiene, Arunas Strumila, Adriana Rozy, Aneta Zdral, Arunas Valiulis

Adv Respir Med. 2024 Aug; 92(4): 291–299. Published online 2024 Aug 5. doi: 10.3390/arm92040028

PMCID:: PMC11351570

Abstract Article PDF–513K

Select item 112528332.

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

Abstract Article PDF–4.6M

Select item 112099353.

Antagonistic roles of canonical and alternative-RPA in disease-associated tandem CAG repeat instability

Terence Gall-Duncan, Jennifer Luo, Carla-Marie Jurkovic, Laura A. Fischer, Kyota Fujita, Amit L. Deshmukh, Rachel J. Harding, Stephanie Tran, Mustafa Mehkary, Vanessa Li, David E. Leib, Ran Chen, Hikari Tanaka, Amanda G. Mason, Dominique Lévesque, Mahreen Khan, Mortezaali Razzaghi, Tanya Prasolava, Stella Lanni, Nozomu Sato, Marie-Christine Caron, Gagan B. Panigrahi, Peixiang Wang, Rachel Lau, Arturo López Castel, Jean-Yves Masson, Lynette Tippett, Clinton Turner, Maria Spies, Albert R. La Spada, Eric I. Campos, Maurice A. Curtis, François-Michel Boisvert, Richard L.M. Faull, Beverly L. Davidson, Masayuki Nakamori, Hitoshi Okazawa, Marc S. Wold, Christopher E. Pearson

Cell. Author manuscript; available in PMC 2024 Oct 26.

Published in final edited form as: Cell. 2023 Oct 26; 186(22): 4898–4919.e25. Published online 2023 Oct 11. doi: 10.1016/j.cell.2023.09.008

PMCID:: PMC11209935

Abstract Article PDF–3.0M

Select item 111208704.

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

Abstract Article PDF–246K

Select item 111140745.

Probing Protein Structural Changes in Alzheimer’s Disease via Quantitative Cross-linking Mass Spectrometry

Zexin Zhu, Xiaofang Zhong, Bin Wang, Haiyan Lu, Lingjun Li

Anal Chem. Author manuscript; available in PMC 2024 May 23.

Published in final edited form as: Anal Chem. 2024 May 14; 96(19): 7506–7515. Published online 2024 May 1. doi: 10.1021/acs.analchem.4c00182

PMCID:: PMC11114074

Abstract Article PDF–1.6M

Select item 110683956.

Alpha-1 antitrypsin inhibits fractalkine-mediated monocyte-lung endothelial cell interactions

Andrew Mikosz, Kevin Ni, Fabienne Gally, Katherine A. Pratte, Seth Winfree, Qiong Lin, Isabelle Echelman, Brianna Wetmore, Danting Cao, Matthew J. Justice, Robert A. Sandhaus, Lisa Maier, Charlie Strange, Russell P. Bowler, Irina Petrache, Karina A. Serban

Am J Physiol Lung Cell Mol Physiol. 2023 Dec 1; 325(6): L711–L725. Published online 2023 Oct 10. doi: 10.1152/ajplung.00023.2023

PMCID:: PMC11068395

Abstract Article

Select item 110085527.

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

Abstract Article PDF–8.1M

Select item 109846558.

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

Abstract Article PDF–252K

Select item 109562549.

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

Abstract Article PDF–1.4M

Select item 1088941310.

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

Abstract Article PDF–4.6M

Select item 1088552311.

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

Abstract Article PDF–3.0M

Select item 1087086612.

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

Abstract Article PDF–1.2M

Select item 1084991513.

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

Abstract Article PDF–1.7M

Select item 1083008614.

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

Abstract Article PDF–1.4M

Select item 1082939815.

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

Abstract Article PDF–1.7M

Select item 1079798516.

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

Abstract Article PDF–1.4M

Select item 1076058617.

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

Abstract Article PDF–658K

Select item 1052497818.

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

Abstract Article PDF–74K

Select item 1049202419.

Increased expression or activation of TRPML1 reduces hepatic storage of toxic Z alpha-1 antitrypsin

Nunzia Pastore, Francesco Annunziata, Rita Colonna, Veronica Maffia, Teresa Giuliano, Bruno Maria Custode, Bernadette Lombardi, Elena Polishchuk, Vincenzo Cacace, Lucia De Stefano, Edoardo Nusco, Nicolina Cristina Sorrentino, Pasquale Piccolo, Nicola Brunetti-Pierri

Mol Ther. 2023 Sep 6; 31(9): 2651–2661. Published online 2023 Jul 1. doi: 10.1016/j.ymthe.2023.06.018

PMCID:: PMC10492024

Abstract Article PDF–4.0M

Select item 1048817220.

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

Abstract Article PDF–3.6M

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