Surfactant protein C (SP-C) is an important player in enhancing the interfacial adsorption of
lung surfactant lipid films to the alveolar air-liquid interface. Doing so, surface tension drops …

Genetic disorders of surfactant production are rare but associated with significant morbidity
and mortality. Diagnosis can be made invasively through clinically available genetic testing …

Interstitial lung disease (ILD) represents a large group of diseases characterized by chronic
inflammation and fibrosis of the lungs, for which therapeutic options are limited. Among …

Interstitial lung disease (ILD) in children (chILD) is a heterogeneous group of rare respiratory
disorders that are mostly chronic and associated with high morbidity and mortality. The …

Abstract BiP co-chaperones ERdj4, ERdj5, and GRP170 associate in cells with peptides
predicted to be aggregation prone. Here, extending these findings to a full-length protein, we …

Pulmonary surfactant is a complex lipoprotein mixture secreted into the alveolar lumen by
type 2 pneumocytes, which is composed by tens of different lipids (approximately 90% of its …

Amyloid fibrils are physiologically insoluble biophysically specific β-sheet rich structures
formed by the aggregation of misfolded proteins. In vivo tissue amyloid formation is …

Genetic factors play an important role in chILD, and the ongoing development of novel
technologies will rapidly broaden the genetic landscape of chILD. Therefore, in the coming …

Although our understanding of the genetics and pathology of congenital lung diseases such
as surfactant protein deficiency, cystic fibrosis, and alpha-1 antitrypsin deficiency is …

There is a wide differential diagnosis of early onset respiratory distress especially in term
babies, and interstitial lung disease (chILD) is a rare but important consideration in this …