Idiopathic pulmonary fibrosis

L Richeldi, HR Collard, MG Jones - The Lancet, 2017 - thelancet.com
Idiopathic pulmonary fibrosis is a prototype of chronic, progressive, and fibrotic lung disease.
Healthy tissue is replaced by altered extracellular matrix and alveolar architecture is …

[HTML][HTML] Exploring animal models that resemble idiopathic pulmonary fibrosis

J Tashiro, GA Rubio, AH Limper, K Williams… - Frontiers in …, 2017 - frontiersin.org
Large multicenter clinical trials have led to two recently approved drugs for patients with
idiopathic pulmonary fibrosis (IPF); yet, both of these therapies only slow disease …

An exome sequencing study to assess the role of rare genetic variation in pulmonary fibrosis

S Petrovski, JL Todd, MT Durheim, Q Wang… - American journal of …, 2017 - atsjournals.org
Rationale: Idiopathic pulmonary fibrosis (IPF) is an increasingly recognized, often fatal lung
disease of unknown etiology. Objectives: The aim of this study was to use whole-exome …

[HTML][HTML] Mitochondria in the spotlight of aging and idiopathic pulmonary fibrosis

AL Mora, M Bueno, M Rojas - The Journal of clinical …, 2017 - Am Soc Clin Investig
Idiopathic pulmonary fibrosis (IPF) is a chronic age-related lung disease with high mortality
that is characterized by abnormal scarring of the lung parenchyma. There has been a recent …

[HTML][HTML] Genetics in idiopathic pulmonary fibrosis pathogenesis, prognosis, and treatment

A Kaur, SK Mathai, DA Schwartz - Frontiers in medicine, 2017 - frontiersin.org
Idiopathic pulmonary fibrosis (IPF), the most common form of idiopathic interstitial
pneumonia (IIP), is characterized by irreversible scarring of the lung parenchyma and …

Tissue remodelling in pulmonary fibrosis

L Knudsen, C Ruppert, M Ochs - Cell and tissue research, 2017 - Springer
Many lung diseases result in fibrotic remodelling. Fibrotic lung disorders can be divided into
diseases with known and unknown aetiology. Among those with unknown aetiology …

[HTML][HTML] Short telomere length in IPF lung associates with fibrotic lesions and predicts survival

R Snetselaar, AA van Batenburg, MFM van Oosterhout… - PloS one, 2017 - journals.plos.org
Telomere maintenance dysfunction has been implicated in the pathogenesis of Idiopathic
Pulmonary Fibrosis (IPF). However, the mechanism of how telomere length is related to …

Regulation of MUC5B Expression in Idiopathic Pulmonary Fibrosis

BA Helling, AN Gerber, V Kadiyala… - American journal of …, 2017 - atsjournals.org
The gain-of-function mucin 5B (MUC5B) promoter variant, rs35705950, confers the largest
risk, genetic or otherwise, for the development of idiopathic pulmonary fibrosis; however, the …

Reactive oxygen species as signaling molecules in the development of lung fibrosis

FJ Gonzalez-Gonzalez, NS Chandel, M Jain… - Translational …, 2017 - Elsevier
Pulmonary fibrosis is a relatively rare but devastating disease characterized by the
excessive deposition of extracellular matrix. The increased matrix results in reduced lung …

[HTML][HTML] Genetic evaluation and testing of patients and families with idiopathic pulmonary fibrosis

JA Kropski, LR Young, JD Cogan… - American journal of …, 2017 - atsjournals.org
There has been increasing recognition that genetic factors play important roles in both
sporadic and familial cases of idiopathic pulmonary fibrosis (IPF)(1). Current data indicate …