纯合子SFTPA1突变驱动特发性肺纤维化患者II型肺泡上皮细胞坏死

The Tokushima journal of experimental medicine Pub Date : 2019-10-10 DOI:10.1084/jem.20182351

Akio Takezaki, S. Tsukumo, Y. Setoguchi, J. Ledford, H. Goto, K. Hosomichi, H. Uehara, Y. Nishioka, K. Yasutomo

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引用次数: 45

摘要

我们在特发性肺纤维化(IPF)患者中发现了SFTPA1的纯合突变。该突变通过IRE1α-JNK轴导致II型肺泡上皮细胞坏死性凋亡增加，这突出了坏死性凋亡途径作为IPF的治疗靶点。

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

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.

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The Tokushima journal of experimental medicine

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