From organ to cell: Multi-level telomere length assessment in patients with idiopathic pulmonary fibrosis.

ABSTRACT:

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 have short telomeres in fibrotic lesions. Our objectives were to better understand how telomere shortening associates with fibrosis in IPF lung and identify a subset of patients with telomere-related disease.

Methods

Average telomere length was determined in multiple organs, basal and apical lung, and diagnostic and end-stage fibrotic lung biopsies. Alveolar type 2 cells telomere length was determined in different areas of IPF lungs.

Results

In IPF but not in controls, telomere length in lung was shorter than in other organs, providing rationale to focus on telomere length in lung. Tel

SUBMITTER: van Batenburg AA 

PROVIDER: S-EPMC6946122 | biostudies-literature | 2020

REPOSITORIES: biostudies-literature