| http://purl.uniprot.org/citations/31601679 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/31601679 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/31601679 | http://www.w3.org/2000/01/rdf-schema#comment | "Idiopathic pulmonary fibrosis (IPF) is a fatal disease characterized by scattered fibrotic lesions in the lungs. The pathogenesis and genetic basis of IPF remain poorly understood. Here, we show that a homozygous missense mutation in SFTPA1 caused IPF in a consanguineous Japanese family. The mutation in SFTPA1 disturbed the secretion of SFTPA1 protein. Sftpa1 knock-in (Sftpa1-KI) mice that harbored the same mutation as patients spontaneously developed pulmonary fibrosis that was accelerated by influenza virus infection. Sftpa1-KI mice showed increased necroptosis of alveolar epithelial type II (AEII) cells with phosphorylation of IRE1α leading to JNK-mediated up-regulation of Ripk3. The inhibition of JNK ameliorated pulmonary fibrosis in Sftpa1-KI mice, and overexpression of Ripk3 in Sftpa1-KI mice treated with a JNK inhibitor worsened pulmonary fibrosis. These findings provide new insight into the mechanisms of IPF in which a mutation in SFTPA1 promotes necroptosis of AEII cells through JNK-mediated up-regulation of Ripk3, highlighting the necroptosis pathway as a therapeutic target for IPF."xsd:string |
| http://purl.uniprot.org/citations/31601679 | http://purl.org/dc/terms/identifier | "doi:10.1084/jem.20182351"xsd:string |
| http://purl.uniprot.org/citations/31601679 | http://purl.org/dc/terms/identifier | "doi:10.1084/jem.20182351"xsd:string |
| http://purl.uniprot.org/citations/31601679 | http://purl.uniprot.org/core/author | "Goto H."xsd:string |
| http://purl.uniprot.org/citations/31601679 | http://purl.uniprot.org/core/author | "Goto H."xsd:string |
| http://purl.uniprot.org/citations/31601679 | http://purl.uniprot.org/core/author | "Hosomichi K."xsd:string |
| http://purl.uniprot.org/citations/31601679 | http://purl.uniprot.org/core/author | "Hosomichi K."xsd:string |
| http://purl.uniprot.org/citations/31601679 | http://purl.uniprot.org/core/author | "Setoguchi Y."xsd:string |
| http://purl.uniprot.org/citations/31601679 | http://purl.uniprot.org/core/author | "Setoguchi Y."xsd:string |
| http://purl.uniprot.org/citations/31601679 | http://purl.uniprot.org/core/author | "Nishioka Y."xsd:string |
| http://purl.uniprot.org/citations/31601679 | http://purl.uniprot.org/core/author | "Nishioka Y."xsd:string |
| http://purl.uniprot.org/citations/31601679 | http://purl.uniprot.org/core/author | "Uehara H."xsd:string |
| http://purl.uniprot.org/citations/31601679 | http://purl.uniprot.org/core/author | "Uehara H."xsd:string |
| http://purl.uniprot.org/citations/31601679 | http://purl.uniprot.org/core/author | "Yasutomo K."xsd:string |
| http://purl.uniprot.org/citations/31601679 | http://purl.uniprot.org/core/author | "Yasutomo K."xsd:string |
| http://purl.uniprot.org/citations/31601679 | http://purl.uniprot.org/core/author | "Ledford J.G."xsd:string |
| http://purl.uniprot.org/citations/31601679 | http://purl.uniprot.org/core/author | "Ledford J.G."xsd:string |
| http://purl.uniprot.org/citations/31601679 | http://purl.uniprot.org/core/author | "Takezaki A."xsd:string |
| http://purl.uniprot.org/citations/31601679 | http://purl.uniprot.org/core/author | "Takezaki A."xsd:string |
| http://purl.uniprot.org/citations/31601679 | http://purl.uniprot.org/core/author | "Tsukumo S.I."xsd:string |
| http://purl.uniprot.org/citations/31601679 | http://purl.uniprot.org/core/author | "Tsukumo S.I."xsd:string |
| http://purl.uniprot.org/citations/31601679 | http://purl.uniprot.org/core/date | "2019"xsd:gYear |
| http://purl.uniprot.org/citations/31601679 | http://purl.uniprot.org/core/date | "2019"xsd:gYear |