PMC:4156421 / 3030-4034 JSONTXT

{"project":"2_test","denotations":[{"id":"25192356-22850886-90446702","span":{"begin":91,"end":92},"obj":"22850886"},{"id":"25192356-11806838-90446703","span":{"begin":417,"end":418},"obj":"11806838"},{"id":"25192356-21506745-90446704","span":{"begin":422,"end":423},"obj":"21506745"},{"id":"25192356-22917154-90446705","span":{"begin":566,"end":567},"obj":"22917154"},{"id":"25192356-23268535-90446706","span":{"begin":570,"end":572},"obj":"23268535"},{"id":"25192356-24429156-90446707","span":{"begin":989,"end":991},"obj":"24429156"},{"id":"25192356-21506748-90446708","span":{"begin":994,"end":996},"obj":"21506748"},{"id":"25192356-23940607-90446709","span":{"begin":1000,"end":1002},"obj":"23940607"}],"text":"Interstitial lung diseases can be difficult to clinically and pathologically characterize [6]. Many cases are diagnosed as idiopathic, highlighting a need to develop better understanding of their pathogenesis. Current evidence suggests that IPF follows a “two-hit” disease model where hereditary factors alter underlying disease susceptibility to environmental stressors such as cigarette smoke, asbestos, or silica [7], [8]. Familial IPF kindred and genome-wide association studies (GWAS) have identified several genetic variants that alter disease susceptibility [9]–[15]. Interestingly, many of the variants described in the literature are known to be involved in host defense, cell adhesion, maintenance of genomic integrity, and preservation of lung architecture. To cite one prominent example, a single nucleotide polymorphism (SNP) in the promoter of the MUC5B gene was recently reported to up-regulate expression of the gene and is both genetically linked and associated with IPF [10]–[14], [16]."}