Frontiers16d
Case report: Infantile pulmonary alveolar proteinosis associated with cytosolic isoleucyl-tRNA synthetase deficiency
Pulmonary alveolar proteinosis (PAP) is a rare phenotype of IARS1 deficiency ... They both experienced respiratory distress and oxygen dependence since infancy. Their chest CT scans revealed diffuse ...
The EMBO Journal28d
A novel human fetal lung-derived alveolar organoid model reveals mechanisms of surfactant protein C maturation relevant to interstitial lung disease
Alveolar type 2 (AT2) cells maintain lung health by acting as stem cells and producing pulmonary surfactant. AT2 dysfunction underlies many lung diseases, including interstitial lung disease (ILD), in ...