Telomere maintenance dysfunction has been implicated in the pathogenesis of Idiopathic
Pulmonary Fibrosis (IPF). However, the mechanism of how telomere length is related to …

Background Telomere shortening has been associated with several lung diseases. However,
telomere length is generally measured in peripheral blood leucocytes rather than in lung …

Rationale A subset of patients with idiopathic pulmonary fibrosis (IPF) contains short leukocyte
telomeres or telomere related mutations. We previously showed that alveolar type 2 cells …

Pulmonary fibrosis is strongly associated with telomere shortening and increased DNA
damage. Key cells in the pathogenesis involve alveolar type 2 (AT2) cells, club cells and …

Background and objective Diagnostic and predictive genetic testing for disease cause and
risk estimation is common in many countries. For genetic diseases, predictive test results are …

Pulmonary fibrosis is the main cause of severe morbidity and mortality in idiopathic interstitial
pneumonias (IIP). In the past years, there has been major progress in the discovery of …

Familial pulmonary fibrosis (FPF) is a monogenic disease most commonly involving telomere-
(TERT) or surfactant- (SFTP) related mutations. These mutations have been shown to alter …

Neutrophils contain high levels of chymotrypsin‐like serine proteases (NSPs) within their
azurophilic granules that have a multitude of functions within the immune system. In response, …

Telomeres are small repetitive DNA sequences at the ends of chromosomes which act as a
buffer in age-dependent DNA shortening. Insufficient telomere repeats will be recognized as …

In only around 40% of families with pulmonary fibrosis (PF) a suspected genetic cause can
be found. Genetic overlap analysis of Whole Exome Sequencing (WES) data may be a …