Association between circulating alpha-1 antitrypsin polymers and lung and liver disease

Núñez, Alexa|||0000-0003-4067-0466; Belmonte, Irene|||0000-0002-8675-8343; Miranda, Elena; Barrecheguren, Miriam|||0000-0002-6041-1499; Farago, Georgina; Loeb, Eduardo; Pons Delgado, Mònica|||0000-0002-0985-3320; Rodríguez Frías, Francisco|||0000-0002-9128-7013; Gabriel-Medina, Pablo|||0000-0003-3079-6364; Rodríguez, Esther|||0000-0001-8572-5198; Genescà Ferrer, Joan|||0000-0002-0831-8422; Miravitlles, Marc|||0000-0002-9850-9520; Esquinas, Cristina|||0000-0001-5568-257X

Association between circulating alpha-1 antitrypsin polymers and lung and liver disease

Alpha-1 antitrypsin deficiency (AATD) is considered one of the most common genetic diseases and is characterised by the misfolding and polymerisation of the alpha-1 antitrypsin (AAT) protein within hepatocytes. The relevance of circulating polymers (CP) of AAT in the pathogenesis of lung and liver d...

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Autores:	Núñez, Alexa\|\|\|0000-0003-4067-0466, Belmonte, Irene\|\|\|0000-0002-8675-8343, Miranda, Elena, Barrecheguren, Miriam\|\|\|0000-0002-6041-1499, Farago, Georgina, Loeb, Eduardo, Pons Delgado, Mònica\|\|\|0000-0002-0985-3320, Rodríguez Frías, Francisco\|\|\|0000-0002-9128-7013, Gabriel-Medina, Pablo\|\|\|0000-0003-3079-6364, Rodríguez, Esther\|\|\|0000-0001-8572-5198, Genescà Ferrer, Joan\|\|\|0000-0002-0831-8422, Miravitlles, Marc\|\|\|0000-0002-9850-9520, Esquinas, Cristina\|\|\|0000-0001-5568-257X
Tipo de recurso:	artículo
Fecha de publicación:	2021
País:	España
Institución:	Universitat Autònoma de Barcelona
Repositorio:	Dipòsit Digital de Documents de la UAB
Idioma:	inglés
OAI Identifier:	oai:dnet:uabarcelona_::f52a3c12a97f9d539cdf25ed8d6247e8
Acceso en línea:	https://ddd.uab.cat/record/327654 https://dx.doi.org/urn:doi:10.1186/s12931-021-01842-5
Access Level:	acceso abierto
Palabra clave:	Alpha-1 antitrypsin deficiency Circulating polymers Emphysema Liver disease

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spelling	Association between circulating alpha-1 antitrypsin polymers and lung and liver diseaseNúñez, Alexa\|\|\|0000-0003-4067-0466Belmonte, Irene\|\|\|0000-0002-8675-8343Miranda, ElenaBarrecheguren, Miriam\|\|\|0000-0002-6041-1499Farago, GeorginaLoeb, EduardoPons Delgado, Mònica\|\|\|0000-0002-0985-3320Rodríguez Frías, Francisco\|\|\|0000-0002-9128-7013Gabriel-Medina, Pablo\|\|\|0000-0003-3079-6364Rodríguez, Esther\|\|\|0000-0001-8572-5198Genescà Ferrer, Joan\|\|\|0000-0002-0831-8422Miravitlles, Marc\|\|\|0000-0002-9850-9520Esquinas, Cristina\|\|\|0000-0001-5568-257XAlpha-1 antitrypsin deficiencyCirculating polymersEmphysemaLiver diseaseAlpha-1 antitrypsin deficiency (AATD) is considered one of the most common genetic diseases and is characterised by the misfolding and polymerisation of the alpha-1 antitrypsin (AAT) protein within hepatocytes. The relevance of circulating polymers (CP) of AAT in the pathogenesis of lung and liver disease is not completely understood. Therefore, the main objective of our study was to determine whether there is an association between the levels of CP of AAT and the severity of lung and liver disease. This was a cross-sectional study in patients with different phenotypes of AATD and controls. To quantify CP, a sandwich ELISA was performed using the 2C1 monoclonal antibody against AAT polymers. Sociodemographic data, clinical characteristics, and liver and lung parameters were collected. A cohort of 70 patients was recruited: 32 PiZZ (11 on augmentation therapy); 29 Z-heterozygous; 9 with other genotypes. CP were compared with a control group of 47 individuals (35 PiMM and 12 Pi*MS). ZZ patients had the highest concentrations of CP (p. 22021-01-0120212021-01-01Articlehttp://purl.org/coar/resource_type/c_6501VoRhttp://purl.org/coar/version/c_970fb48d4fbd8a85info:eu-repo/semantics/articleapplication/pdfhttps://ddd.uab.cat/record/327654https://dx.doi.org/urn:doi:10.1186/s12931-021-01842-5reponame:Dipòsit Digital de Documents de la UABinstname:Universitat Autònoma de BarcelonaInglésengopen accesshttp://purl.org/coar/access_right/c_abf2Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, fins i tot amb finalitats comercials, sempre i quan es reconegui l'autoria de l'obra original.https://creativecommons.org/licenses/by/4.0/info:eu-repo/semantics/openAccessoai:dnet:uabarcelona_::f52a3c12a97f9d539cdf25ed8d6247e82026-06-06T12:50:31Z
dc.title.none.fl_str_mv	Association between circulating alpha-1 antitrypsin polymers and lung and liver disease
title	Association between circulating alpha-1 antitrypsin polymers and lung and liver disease
spellingShingle	Association between circulating alpha-1 antitrypsin polymers and lung and liver disease Núñez, Alexa\|\|\|0000-0003-4067-0466 Alpha-1 antitrypsin deficiency Circulating polymers Emphysema Liver disease
title_short	Association between circulating alpha-1 antitrypsin polymers and lung and liver disease
title_full	Association between circulating alpha-1 antitrypsin polymers and lung and liver disease
title_fullStr	Association between circulating alpha-1 antitrypsin polymers and lung and liver disease
title_full_unstemmed	Association between circulating alpha-1 antitrypsin polymers and lung and liver disease
title_sort	Association between circulating alpha-1 antitrypsin polymers and lung and liver disease
dc.creator.none.fl_str_mv	Núñez, Alexa\|\|\|0000-0003-4067-0466 Belmonte, Irene\|\|\|0000-0002-8675-8343 Miranda, Elena Barrecheguren, Miriam\|\|\|0000-0002-6041-1499 Farago, Georgina Loeb, Eduardo Pons Delgado, Mònica\|\|\|0000-0002-0985-3320 Rodríguez Frías, Francisco\|\|\|0000-0002-9128-7013 Gabriel-Medina, Pablo\|\|\|0000-0003-3079-6364 Rodríguez, Esther\|\|\|0000-0001-8572-5198 Genescà Ferrer, Joan\|\|\|0000-0002-0831-8422 Miravitlles, Marc\|\|\|0000-0002-9850-9520 Esquinas, Cristina\|\|\|0000-0001-5568-257X
author	Núñez, Alexa\|\|\|0000-0003-4067-0466
author_facet	Núñez, Alexa\|\|\|0000-0003-4067-0466 Belmonte, Irene\|\|\|0000-0002-8675-8343 Miranda, Elena Barrecheguren, Miriam\|\|\|0000-0002-6041-1499 Farago, Georgina Loeb, Eduardo Pons Delgado, Mònica\|\|\|0000-0002-0985-3320 Rodríguez Frías, Francisco\|\|\|0000-0002-9128-7013 Gabriel-Medina, Pablo\|\|\|0000-0003-3079-6364 Rodríguez, Esther\|\|\|0000-0001-8572-5198 Genescà Ferrer, Joan\|\|\|0000-0002-0831-8422 Miravitlles, Marc\|\|\|0000-0002-9850-9520 Esquinas, Cristina\|\|\|0000-0001-5568-257X
author_role	author
author2	Belmonte, Irene\|\|\|0000-0002-8675-8343 Miranda, Elena Barrecheguren, Miriam\|\|\|0000-0002-6041-1499 Farago, Georgina Loeb, Eduardo Pons Delgado, Mònica\|\|\|0000-0002-0985-3320 Rodríguez Frías, Francisco\|\|\|0000-0002-9128-7013 Gabriel-Medina, Pablo\|\|\|0000-0003-3079-6364 Rodríguez, Esther\|\|\|0000-0001-8572-5198 Genescà Ferrer, Joan\|\|\|0000-0002-0831-8422 Miravitlles, Marc\|\|\|0000-0002-9850-9520 Esquinas, Cristina\|\|\|0000-0001-5568-257X
author2_role	author author author author author author author author author author author author
dc.subject.none.fl_str_mv	Alpha-1 antitrypsin deficiency Circulating polymers Emphysema Liver disease
topic	Alpha-1 antitrypsin deficiency Circulating polymers Emphysema Liver disease
description	Alpha-1 antitrypsin deficiency (AATD) is considered one of the most common genetic diseases and is characterised by the misfolding and polymerisation of the alpha-1 antitrypsin (AAT) protein within hepatocytes. The relevance of circulating polymers (CP) of AAT in the pathogenesis of lung and liver disease is not completely understood. Therefore, the main objective of our study was to determine whether there is an association between the levels of CP of AAT and the severity of lung and liver disease. This was a cross-sectional study in patients with different phenotypes of AATD and controls. To quantify CP, a sandwich ELISA was performed using the 2C1 monoclonal antibody against AAT polymers. Sociodemographic data, clinical characteristics, and liver and lung parameters were collected. A cohort of 70 patients was recruited: 32 PiZZ (11 on augmentation therapy); 29 Z-heterozygous; 9 with other genotypes. CP were compared with a control group of 47 individuals (35 PiMM and 12 Pi*MS). ZZ patients had the highest concentrations of CP (p.
publishDate	2021
dc.date.none.fl_str_mv	2 2021-01-01 2021 2021-01-01
dc.type.none.fl_str_mv	Article http://purl.org/coar/resource_type/c_6501 VoR http://purl.org/coar/version/c_970fb48d4fbd8a85
dc.type.openaire.fl_str_mv	info:eu-repo/semantics/article
format	article
dc.identifier.none.fl_str_mv	https://ddd.uab.cat/record/327654 https://dx.doi.org/urn:doi:10.1186/s12931-021-01842-5
url	https://ddd.uab.cat/record/327654 https://dx.doi.org/urn:doi:10.1186/s12931-021-01842-5
dc.language.none.fl_str_mv	Inglés eng
language_invalid_str_mv	Inglés
language	eng
dc.rights.none.fl_str_mv	open access http://purl.org/coar/access_right/c_abf2 https://creativecommons.org/licenses/by/4.0/
dc.rights.openaire.fl_str_mv	info:eu-repo/semantics/openAccess
rights_invalid_str_mv	open access http://purl.org/coar/access_right/c_abf2 https://creativecommons.org/licenses/by/4.0/
eu_rights_str_mv	openAccess
dc.format.none.fl_str_mv	application/pdf
dc.source.none.fl_str_mv	reponame:Dipòsit Digital de Documents de la UAB instname:Universitat Autònoma de Barcelona
instname_str	Universitat Autònoma de Barcelona
reponame_str	Dipòsit Digital de Documents de la UAB
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Association between circulating alpha-1 antitrypsin polymers and lung and liver disease

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