ERN1
Serine/threonine-protein kinase and endoribonuclease that acts as a key sensor for the endoplasmic reticulum unfolded protein response (UPR) (PubMed:11779464, PubMed:11175748, PubMed:12637535, PubMed:9637683, PubMed:21317875). In unstressed cells, the endoplasmic reticulum luminal domain is maintain...
Serine/threonine-protein kinase and endoribonuclease that acts as a key sensor for the endoplasmic reticulum unfolded protein response (UPR) (PubMed:11779464, PubMed:11175748, PubMed:12637535, PubMed:9637683, PubMed:21317875). In unstressed cells, the endoplasmic reticulum luminal domain is maintained in its inactive monomeric state by binding to the endoplasmic reticulum chaperone HSPA5/BiP (PubMed:21317875). Accumulation of misfolded protein in the endoplasmic reticulum causes release of HSPA5/BiP, allowing the luminal domain to homodimerize, promoting autophosphorylation of the kinase domain and subsequent activation of the endoribonuclease activity (PubMed:21317875). The endoribonuclease activity is specific for XBP1 mRNA and excises 26 nucleotides from XBP1 mRNA (PubMed:11779464, PubMed:24508390, PubMed:21317875). The resulting spliced transcript of XBP1 encodes a transcriptional activator protein that up-regulates expression of UPR target genes (PubMed:11779464, PubMed:24508390, PubMed:21317875). Acts as an upstream signal for ER stress-induced GORASP2-mediated unconventional (ER/Golgi-independent) trafficking of CFTR to cell membrane by modulating the expression and localization of SEC16A (PubMed:21884936, PubMed:28067262).
View more >>
GO - Biological processes (BP):
activation of JUN kinase activity, cell cycle arrest, cellular response to glucose stimulus, cellular response to unfolded protein, cellular response to vascular endothelial growth factor stimulus, endoplasmic reticulum unfolded protein response, endothelial cell proliferation, insulin metabolic pro...
activation of JUN kinase activity, cell cycle arrest, cellular response to glucose stimulus, cellular response to unfolded protein, cellular response to vascular endothelial growth factor stimulus, endoplasmic reticulum unfolded protein response, endothelial cell proliferation, insulin metabolic process, intrinsic apoptotic signaling pathway in response to endoplasmic reticulum stress, IRE1-mediated unfolded protein response, mRNA catabolic process, mRNA cleavage, mRNA cleavage involved in mRNA processing, mRNA splicing, via endonucleolytic cleavage and ligation, peptidyl-serine autophosphorylation, peptidyl-serine trans-autophosphorylation, positive regulation of endoplasmic reticulum unfolded protein response, positive regulation of RNA splicing, positive regulation of vascular smooth muscle cell proliferation, protein autophosphorylation, protein phosphorylation, regulation of macroautophagy, response to endoplasmic reticulum stress
View more >>
GO - Molecular function (MF):
ADP binding, ATP binding, endoribonuclease activity, enzyme binding, Hsp70 protein binding, Hsp90 protein binding, identical protein binding, magnesium ion binding, platelet-derived growth factor receptor binding, protein homodimerization activity, protein kinase activity, protein serine/threonine k...
ADP binding, ATP binding, endoribonuclease activity, enzyme binding, Hsp70 protein binding, Hsp90 protein binding, identical protein binding, magnesium ion binding, platelet-derived growth factor receptor binding, protein homodimerization activity, protein kinase activity, protein serine/threonine kinase activity, unfolded protein binding
View more >>
GO - Cellular component (CC):
endoplasmic reticulum, endoplasmic reticulum membrane, integral component of endoplasmic reticulum membrane, Ire1 complex, IRE1-TRAF2-ASK1 complex, mitochondrion, nuclear inner membrane, AIP1-IRE1 complex, cell, cytoplasm, IRE1-RACK1-PP2A complex...
endoplasmic reticulum, endoplasmic reticulum membrane, integral component of endoplasmic reticulum membrane, Ire1 complex, IRE1-TRAF2-ASK1 complex, mitochondrion, nuclear inner membrane, AIP1-IRE1 complex, cell, cytoplasm, IRE1-RACK1-PP2A complex
View more >>