ERN1

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.