Genetic Risk Factors for Idiopathic Pulmonary Fibrosis: Insights into Immunopathogenesis

Jacob E Michalski, David A Schwartz Department of Medicine, University of Colorado School of Medicine, Aurora, CO, USACorrespondence: David A SchwartzUniversity of Colorado School of Medicine, 12631 East 17th Avenue, B178, Aurora, CO 80045, USATel +1 303-724-1783Fax +1 303-724-1799Email david.schwar...

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Autores principales:	Michalski JE, Schwartz DA
Formato:	article
Lenguaje:	EN
Publicado:	Dove Medical Press 2021
Materias:	pulmonary fibrosis interstitial lung disease genetics muc5b host defense innate immunity Pathology RB1-214 Therapeutics. Pharmacology RM1-950
Acceso en línea:	https://doaj.org/article/a2552b8d6e354ff699a30fdd71c59798
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spelling	oai:doaj.org-article:a2552b8d6e354ff699a30fdd71c597982021-12-02T15:15:25ZGenetic Risk Factors for Idiopathic Pulmonary Fibrosis: Insights into Immunopathogenesis1178-7031https://doaj.org/article/a2552b8d6e354ff699a30fdd71c597982021-01-01T00:00:00Zhttps://www.dovepress.com/genetic-risk-factors-for-idiopathic-pulmonary-fibrosis-insights-into-i-peer-reviewed-article-JIRhttps://doaj.org/toc/1178-7031Jacob E Michalski, David A Schwartz Department of Medicine, University of Colorado School of Medicine, Aurora, CO, USACorrespondence: David A SchwartzUniversity of Colorado School of Medicine, 12631 East 17th Avenue, B178, Aurora, CO 80045, USATel +1 303-724-1783Fax +1 303-724-1799Email david.schwartz@ucdenver.eduAbstract: Idiopathic pulmonary fibrosis is an etiologically complex interstitial lung disease characterized by progressive scarring of the lungs with a subsequent decline in lung function. While much of the pathogenesis of IPF still remains unclear, it is now understood that genetic variation accounts for at least one-third of the risk of developing the disease. The single-most validated and most significant risk factor, genetic or otherwise, is a gain-of-function promoter variant in the MUC5B gene. While the functional impact of these IPF risk variants at the cellular and tissue levels are areas of active investigation, there is a growing body of evidence that these genetic variants may influence disease pathogenesis through modulation of innate immune processes.Keywords: pulmonary fibrosis, interstitial lung disease, genetics, MUC5B, host defense, innate immunityMichalski JESchwartz DADove Medical Pressarticlepulmonary fibrosisinterstitial lung diseasegeneticsmuc5bhost defenseinnate immunityPathologyRB1-214Therapeutics. PharmacologyRM1-950ENJournal of Inflammation Research, Vol Volume 13, Pp 1305-1318 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	pulmonary fibrosis interstitial lung disease genetics muc5b host defense innate immunity Pathology RB1-214 Therapeutics. Pharmacology RM1-950
spellingShingle	pulmonary fibrosis interstitial lung disease genetics muc5b host defense innate immunity Pathology RB1-214 Therapeutics. Pharmacology RM1-950 Michalski JE Schwartz DA Genetic Risk Factors for Idiopathic Pulmonary Fibrosis: Insights into Immunopathogenesis
description	Jacob E Michalski, David A Schwartz Department of Medicine, University of Colorado School of Medicine, Aurora, CO, USACorrespondence: David A SchwartzUniversity of Colorado School of Medicine, 12631 East 17th Avenue, B178, Aurora, CO 80045, USATel +1 303-724-1783Fax +1 303-724-1799Email david.schwartz@ucdenver.eduAbstract: Idiopathic pulmonary fibrosis is an etiologically complex interstitial lung disease characterized by progressive scarring of the lungs with a subsequent decline in lung function. While much of the pathogenesis of IPF still remains unclear, it is now understood that genetic variation accounts for at least one-third of the risk of developing the disease. The single-most validated and most significant risk factor, genetic or otherwise, is a gain-of-function promoter variant in the MUC5B gene. While the functional impact of these IPF risk variants at the cellular and tissue levels are areas of active investigation, there is a growing body of evidence that these genetic variants may influence disease pathogenesis through modulation of innate immune processes.Keywords: pulmonary fibrosis, interstitial lung disease, genetics, MUC5B, host defense, innate immunity
format	article
author	Michalski JE Schwartz DA
author_facet	Michalski JE Schwartz DA
author_sort	Michalski JE
title	Genetic Risk Factors for Idiopathic Pulmonary Fibrosis: Insights into Immunopathogenesis
title_short	Genetic Risk Factors for Idiopathic Pulmonary Fibrosis: Insights into Immunopathogenesis
title_full	Genetic Risk Factors for Idiopathic Pulmonary Fibrosis: Insights into Immunopathogenesis
title_fullStr	Genetic Risk Factors for Idiopathic Pulmonary Fibrosis: Insights into Immunopathogenesis
title_full_unstemmed	Genetic Risk Factors for Idiopathic Pulmonary Fibrosis: Insights into Immunopathogenesis
title_sort	genetic risk factors for idiopathic pulmonary fibrosis: insights into immunopathogenesis
publisher	Dove Medical Press
publishDate	2021
url	https://doaj.org/article/a2552b8d6e354ff699a30fdd71c59798
work_keys_str_mv	AT michalskije geneticriskfactorsforidiopathicpulmonaryfibrosisinsightsintoimmunopathogenesis AT schwartzda geneticriskfactorsforidiopathicpulmonaryfibrosisinsightsintoimmunopathogenesis
_version_	1718387525957976064

Genetic Risk Factors for Idiopathic Pulmonary Fibrosis: Insights into Immunopathogenesis

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