Inhibition of EMC3 or VCP restored alveolar morphology in vivo and restored homeostasis in patient-specific induced AT2 (iAT2) cells derived from SFTPCI73T patient-specific induced pluripotent stem cells (iPSCs), supporting the concept that EMC3, VCP, and other components of the EMC complex provide a framework for development of new therapies for SFTPCI73T-associated ILD and other disorders caused by similar mistrafficking of variant proteins. Here, EMC3 is linked to interstitial lung disease.