Therefore, based on these cell culture data, disordered lipid composition, reduced and faulty lamellar body organization due to the G964D – ABCA3 mutation may represent mechanisms involved in the pathogenesis on ILD in this family, whereas misfolding, aberrant processing, mislocalization and ER-stress can be excluded as factors contributing to the development of ILD by the G964D mutation. This evidence concerns the gene ABCA3 and interstitial lung disease.