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ERN1/IRE1α is a key sensor of ER stress and a central player in the unfolded protein response (UPR). It possesses both kinase and RNase activity, which are activated upon accumulation of misfolded proteins in the ER. Activated IRE1α splices XBP1 mRNA, leading to the production of XBP1s, a transcription factor that upregulates genes involved in restoring ER homeostasis. Dysregulation of IRE1α is implicated in several diseases, including neurodegenerative disorders and cancer.
Kinase and RNase activity regulating the UPR pathway, specifically splicing XBP1 mRNA.
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