Indeed, the surfactant protein C (SP-C) gene mutations have been found mostly in familial forms of both IPF and NSIP, together with an increase in the production of misfolded proteins, leading to proteasome inhibition and endoplasmic reticulum (ER) stress that culminate in AECII depletion [102]. The gene discussed is SFTPC; the disease is idiopathic interstitial pneumonia.