ABCA3 protein retention in the endoplasmic reticulum may increase reticulum endoplasmic stress and its susceptibility to stress; an adverse effect of R280C on LB biogenesis and the induced presence of apoptotic markers (glutathione on caspase 4 pathway) were demonstrated in the experimental epithelial lung cells in Weichert et al. experiments [91], which is also suggestive of functional impairment on ABCA3 protein and lung disease pathogenesis. Here, ABCA3 is linked to lung disorder.