ERN1

(redirected from Inositol-requiring protein 1)

ERN1

A gene on chromosome 17q24.2 that encodes the ubiquitously expressed endoplasmic reticulum (ER)-to-nucleus signalling 1 protein, which has intrinsic kinase and endoribonuclease activities and plays a key role in altering gene expression in response to endoplasmic reticulum-based stress signals. ERN1 senses unfolded proteins in the ER lumen, leading to enzyme auto-activation; the active endoribonuclease domain splices XBP1 mRNA, generating a new C-terminus and converting it into a potent unfolded-protein response transcriptional activator, triggering growth arrest and apoptosis.
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Three ER-resident proteins have been identified as sensors of ER stress: IRE1 (inositol-requiring protein 1), PERK (PKR- (double-stranded RNA-dependent protein kinase) like ER kinase), and ATF6 (activating transcription factor) [11].
PERK, inositol-requiring protein 1 (IRE1), and activating transcription factor-6 (ATF-6) are three specialized stress sensors located in the ER membrane.