Fibrosis is one of the hallmarks documented in ABCA3-associated ILD [12,16,17] and knowing that ABCA3 mutations can cause ER retention of the mutated transporter [6,20], we investigated the influence of three ABCA3 mutations, R43L, R280C and L101P, found in children with surfactant deficiency and chronic ILD [10,14,19], on ER stress and apoptosis induction in lung epithelial A549 cells. This evidence concerns the gene ABCA3 and interstitial lung disease.