Host: The Japanese Society of Toxicology
Pulmonary fibrosis is characterized by progressive fibrosis leading to respiratory failure. Our research group has identified a missense mutation in SFTPA1 as a causative mutation in familial pulmonary fibrosis, which impairs the secretion of SFTPA1 protein and is considered to be the starting point of the disease. Functional analysis using Sftap11-KI mice revealed that necroptosis of type II alveolar epithelial cells is the initial cellular event of the disease. In this presentation, we would like to discuss how necroptosis leads to pulmonary fibrosis by using Sftpa1-KI mice.
