The accumulation of misfolded proteins in the endoplasmic reticulum (ER) leads to ER
stress, resulting in activation of the unfolded protein response (UPR) that aims to restore …

With the global increase of the elderly population, the improvement of the treatment for
various aging-related diseases and the extension of a healthy lifespan have become some …

Interstitial lung diseases (ILD) or diffuse parenchymal lung diseases (DPLD) comprise a
large number of disorders. Disease definition and classification allow advanced and …

Autoimmune pulmonary alveolar proteinosis (PAP) is a rare disease characterized by
myeloid cell dysfunction, abnormal pulmonary surfactant accumulation, and innate immune …

Rationale: Genetic studies of idiopathic pulmonary fibrosis (IPF) have improved our
understanding of this disease, but not all causal loci have been identified. Objectives: To …

Idiopathic pulmonary fibrosis (IPF) is a rare disease of the lung with a largely unknown
etiology and a poor prognosis. Intriguingly, forms of familial pulmonary fibrosis (FPF) have …

Patients with familial pulmonary fibrosis represent a subset of patients with pulmonary
fibrosis in whom inherited gene variation predisposes them to disease development. In the …

Disruption of alveolar type 2 cell (AEC2) protein quality control has been implicated in
chronic lung diseases, including pulmonary fibrosis (PF). We previously reported the in vivo …

Background Whole genome sequencing (WGS) can detect variants and estimate telomere
length. The clinical utility of WGS in estimating risk, progression and survival of pulmonary …

Pulmonary surfactant is critically important to prevent atelectasis by lowering the surface
tension of the alveolar lining liquid. While respiratory distress syndrome (RDS) is common in …