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Article

Disruption of proteostasis causes IRE1 mediated reprogramming of alveolar epithelial cells

17 Oct 2022

Abstract excerpt

Disruption of alveolar type 2 cell (AEC2) protein quality control has been implicated in chronic lung diseases, including pulmonary fibrosis (PF). We previously reported the in vivo modeling of a clinical surfactant protein C (SP-C) mutation that led to AEC2 endoplasmic reticulum (ER) stress and spontaneous lung fibrosis, providing proof of concept for disruption to proteostasis as a proximal driver of PF. Using...

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PMID: 36252035
DOI: 10.1073/pnas.2123187119

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