CiliOPD: a ciliopathy-associated COPD endotype

Respir Res. 2021 Feb 27;22(1):74. doi: 10.1186/s12931-021-01665-4.

Abstract

The pathophysiology of chronic obstructive pulmonary disease (COPD) relies on airway remodelling and inflammation. Alterations of mucociliary clearance are a major hallmark of COPD caused by structural and functional cilia abnormalities. Using transcriptomic databases of whole lung tissues and isolated small airway epithelial cells (SAEC), we comparatively analysed cilia-associated and ciliopathy-associated gene signatures from a set of 495 genes in 7 datasets including 538 non-COPD and 508 COPD patients. This bio-informatics approach unveils yet undescribed cilia and ciliopathy genes associated with COPD including NEK6 and PROM2 that may contribute to the pathology, and suggests a COPD endotype exhibiting ciliopathy features (CiliOPD).

Keywords: COPD; Cilia; Transcriptomic.

Publication types

  • Letter

MeSH terms

  • Ciliopathies / diagnosis
  • Ciliopathies / epidemiology
  • Ciliopathies / genetics*
  • Databases, Genetic*
  • Humans
  • Pulmonary Disease, Chronic Obstructive / diagnosis
  • Pulmonary Disease, Chronic Obstructive / epidemiology
  • Pulmonary Disease, Chronic Obstructive / genetics*
  • Sequence Analysis, RNA / methods*