A homozygous SFTPA1 mutation drives necroptosis of type II alveolar epithelial cells in patients with idiopathic pulmonary fibrosis

Abstract

We identify a homozygous mutation in SFTPA1 in patients with idiopathic pulmonary fibrosis (IPF). The mutation causes increased necroptosis of type II alveolar epithelial cells through the IRE1α–JNK axis, which highlights the necroptosis pathway as a therapeutic target for IPF.