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
Idiopathic pulmonary fibrosis (IPF) is an incurable complex genetic disorder that is
associated with sequence changes in 7 genes (MUC5B, TERT, TERC, RTEL1, PARN …

MUC5B and idiopathic pulmonary fibrosis

IV Yang, TE Fingerlin, CM Evans… - Annals of the …, 2015 - atsjournals.org
Idiopathic pulmonary fibrosis (IPF), a fatal disease that is a result of complex interactions
between genetics and the environment, has limited treatment options. We have identified the …

Genetics and early detection in idiopathic pulmonary fibrosis

RK Putman, IO Rosas… - American journal of …, 2014 - atsjournals.org
Genetic studies hold promise in helping to identify patients with early idiopathic pulmonary
fibrosis (IPF). Recent studies using chest computed tomograms (CTs) in smokers and in the …

Genetic risk factors for idiopathic pulmonary fibrosis: insights into immunopathogenesis

JE Michalski, DA Schwartz - Journal of Inflammation Research, 2021 - Taylor & Francis
Idiopathic pulmonary fibrosis is an etiologically complex interstitial lung disease
characterized by progressive scarring of the lungs with a subsequent decline in lung …

Pulmonary fibrosis distal airway epithelia are dynamically and structurally dysfunctional

IT Stancil, JE Michalski, D Davis-Hall, HW Chu… - Nature …, 2021 - nature.com
The airway epithelium serves as the interface between the host and external environment. In
many chronic lung diseases, the airway is the site of substantial remodeling after injury …

MUC5B Promoter Variant rs35705950 Affects MUC5B Expression in the Distal Airways in Idiopathic Pulmonary Fibrosis

Y Nakano, IV Yang, AD Walts, AM Watson… - American journal of …, 2016 - atsjournals.org
Results We identified 16 variants in the 4.2-kb 5′ promoter region of MUC5B within the six
GG and six TT study subjects with IPF. Each subject had a unique haplotype for the putative …

A Variant in the Promoter of MUC5B and Idiopathic Pulmonary Fibrosis

Y Zhang, I Noth, JGN Garcia… - New England Journal of …, 2011 - Mass Medical Soc
A Variant in the Promoter of <italic>MUC5B</italic> and Idiopathic Pulmonary Fibrosis Page 1
n engl j med 364;16 nejm.org april 21, 2011 1576 The new england journal of medicine …

Molecular mechanisms in progressive idiopathic pulmonary fibrosis

MP Steele, DA Schwartz - Annual review of medicine, 2013 - annualreviews.org
There is clear evidence that environmental exposures and genetic predisposition contribute
to the pathogenesis of idiopathic pulmonary fibrosis (IPF). Cigarette smoking increases the …

Pulmonary fibrosis in the era of stratified medicine

SK Mathai, CA Newton, DA Schwartz, CK Garcia - Thorax, 2016 - thorax.bmj.com
Both common and rare variants contribute to the genetic architecture of pulmonary fibrosis.
Genome-wide association studies have identified common variants, or those with a minor …

The idiopathic pulmonary fibrosis honeycomb cyst contains a mucocilary pseudostratified epithelium

MA Seibold, RW Smith, C Urbanek, SD Groshong… - PloS one, 2013 - journals.plos.org
Background We previously identified a MUC5B gene promoter-variant that is a risk allele for
sporadic and familial Idiopathic Pulmonary Fibrosis/Usual Interstitial Pneumonia (IPF/UIP) …