[HTML][HTML] Idiopathic pulmonary fibrosis is a complex genetic disorder

DA Schwartz - Transactions of the American Clinical and …, 2016 - ncbi.nlm.nih.gov
idiopathic pulmonary fibrosis and we’re in the process of studying the relationship between
cigarette smoking, excess MUCB production, and the development of pulmonary fibrosis. In …

The genetic basis of idiopathic pulmonary fibrosis

JA Kropski, TS Blackwell… - European Respiratory …, 2015 - Eur Respiratory Soc
… Notably, the short telomere phenotype in peripheral blood mononuclear cells (PBMCs) is
not limited to patients with loss of function rare variants in telomerase complex genes. …

Idiopathic pulmonary fibrosis: a genetic disease that involves mucociliary dysfunction of the peripheral airways

CM Evans, TE Fingerlin, MI Schwarz… - Physiological …, 2016 - journals.physiology.org
… a complex genetic disorder (189), suggesting that the different histological types of idiopathic
… These findings highlight the complex nature of IPF and suggest that while a susceptibility …

Genetic studies provide clues on the pathogenesis of idiopathic pulmonary fibrosis

JA Kropski, WE Lawson, LR Young… - Disease models & …, 2013 - journals.biologists.com
… -causing heterozygous mutations in TERT (encoding telomerase reverse transcriptase)
and TERC (encoding the RNA component of the telomerase complex). Notably, …

Idiopathic pulmonary fibrosis

FJ Martinez, HR Collard, A Pardo, G Raghu… - Nature reviews Disease …, 2017 - nature.com
… -third of the inherent individual risk of disease 23 . Age is the … Indeed, IPF is a complex disorder
resulting from interactions … , suggesting a complex deregulation of downstream genes 54 . …

Idiopathic pulmonary fibrosis: a disorder of epithelial cell dysfunction

DF Zoz, WE Lawson, TS Blackwell - The American journal of the medical …, 2011 - Elsevier
… AECs in pulmonary fibrosis comes from studies in the past decade linking genetic mutations
… explants usually show end-stage fibrosis, and it is difficult to determine the primary inciting …

Molecular mechanisms in progressive idiopathic pulmonary fibrosis

MP Steele, DA Schwartz - Annual review of medicine, 2013 - annualreviews.org
Genetic predisposition to IPF is evident from its familial aggregation and the fact that pulmonary
fibrosis develops in several rare genetic disorderscomplex disease with multiple genetic

Idiopathic pulmonary fibrosis beyond the lung: understanding disease mechanisms to improve diagnosis and management

F Luppi, M Kalluri, P Faverio, M Kreuter, G Ferrara - Respiratory Research, 2021 - Springer
… and sporadic pulmonary fibrosis has arisen from the recent description of germline
mutations in the genes hTERT and hTR, which are associated with the telomerase complex [32]. …

Idiopathic pulmonary fibrosis: pathogenesis and management

G Sgalla, B Iovene, M Calvello, M Ori, F Varone… - Respiratory …, 2018 - Springer
… 9-DPP9) for the development of the disease. Both the GWAS established the role of the
promoter of MUC5B gene as a risk factor of disease and characterized other common variants …

Idiopathic pulmonary fibrosis: update on genetic discoveries

CK Garcia - Proceedings of the American Thoracic Society, 2011 - atsjournals.org
diseases (ILDs) are a heterogeneous collection of over 100 different diseases, all characterized
by pulmonary fibrosis. … Unlike cystic fibrosis, the genetics of IPF are complex. There is …