ERN1


Summary: This gene encodes the transmembrane protein kinase inositol-requiring enzyme 1. The encoded protein contains two functional catalytic domains, a serine/threonine-protein kinase domain and an endoribonuclease domain. This protein functions as a sensor of unfolded proteins in the endoplasmic reticulum (ER) and triggers an intracellular signaling pathway termed the unfolded protein response (UPR). The UPR is an ER stress response that is conserved from yeast to mammals and activates genes involved in degrading misfolded proteins, regulating protein synthesis and activating molecular chaperones. This protein specifically mediates the splicing and activation of the stress response transcription factor X-box binding protein 1. [provided by RefSeq, Aug 2017].

NameOMIM IDEnsembl IDHGNC IDPHARMGKB IDMap Location
endoplasmic reticulum to nucleus signaling 1MIM:604033Ensembl:ENSG00000178607HGNC:HGNC:3449PA2786117q23.3

GO terms in ERN1


Term TypeEvidence TypeGO Term IDGO Des.
MFIDAGO:0000287magnesium ion binding
MFIDAGO:0004521endoribonuclease activity
MFTASGO:0004521endoribonuclease activity
MFIDAGO:0004674protein serine/threonine kinase activity
MFTASGO:0004674protein serine/threonine kinase activity
MFIPIGO:0005161platelet-derived growth factor receptor binding
MFIPIGO:0005515protein binding
MFIDAGO:0005524ATP binding
MFIPIGO:0019899enzyme binding
MFIPIGO:0030544Hsp70 protein binding
MFIPIGO:0042802identical protein binding
MFIDAGO:0042803protein homodimerization activity
MFISSGO:0042803protein homodimerization activity
MFIDAGO:0043531ADP binding
MFIDAGO:0051879Hsp90 protein binding
CCIEAGO:0005637nuclear inner membrane
CCIDAGO:0005737cytoplasm
CCIEAGO:0005739mitochondrion
CCIDAGO:0005783endoplasmic reticulum
CCTASGO:0005789endoplasmic reticulum membrane
CCIDAGO:0030176integral component of endoplasmic reticulum membrane
CCNASGO:1990332Ire1 complex
CCIEAGO:1990597AIP1-IRE1 complex
CCIDAGO:1990604IRE1-TRAF2-ASK1 complex
CCIDAGO:1990630IRE1-RACK1-PP2A complex
BPIDAGO:0001935endothelial cell proliferation
BPIEAGO:0006351transcription, DNA-templated
BPIEAGO:0006355regulation of transcription, DNA-templated
BPISSGO:0006379mRNA cleavage
BPTASGO:0006402mRNA catabolic process
BPIDAGO:0006468protein phosphorylation
BPISSGO:0007050cell cycle arrest
BPIDAGO:0007257activation of JUN kinase activity
BPTASGO:0016241regulation of macroautophagy
BPIDAGO:0033120positive regulation of RNA splicing
BPIDAGO:0034620cellular response to unfolded protein
BPIMPGO:0034620cellular response to unfolded protein
BPIDAGO:0034976response to endoplasmic reticulum stress
BPIDAGO:0035924cellular response to vascular endothelial growth factor stimulus
BPIDAGO:0036289peptidyl-serine autophosphorylation
BPIDAGO:0036498IRE1-mediated unfolded protein response
BPTASGO:0036498IRE1-mediated unfolded protein response
BPIDAGO:0046777protein autophosphorylation
BPIDAGO:0070054mRNA splicing, via endonucleolytic cleavage and ligation
BPIMPGO:0070054mRNA splicing, via endonucleolytic cleavage and ligation
BPTASGO:0070054mRNA splicing, via endonucleolytic cleavage and ligation
BPIEAGO:0070059intrinsic apoptotic signaling pathway in response to endoplasmic reticulum stress
BPIDAGO:0071333cellular response to glucose stimulus
BPIEAGO:0090502RNA phosphodiester bond hydrolysis, endonucleolytic
BPIDAGO:0098787mRNA cleavage involved in mRNA processing
BPIMPGO:1900103positive regulation of endoplasmic reticulum unfolded protein response
BPIDAGO:1901142insulin metabolic process
BPIMPGO:1904707positive regulation of vascular smooth muscle cell proliferation
BPIMPGO:1990579peptidyl-serine trans-autophosphorylation

Gene expression in normal tissue: ERN1

Gene-model tissue-cancer distribution: Bubble Plot

Gene-drug pathway distribution

Pathways in ERN1


DatabasePathway IDPathway Des.
reactomeR-HSA-381070IRE1alpha activates chaperones
reactomeR-HSA-381070IRE1alpha activates chaperones
reactomeR-HSA-381119Unfolded Protein Response (UPR)
reactomeR-HSA-381119Unfolded Protein Response (UPR)
reactomeR-HSA-392499Metabolism of proteins
reactomeR-HSA-392499Metabolism of proteins
kegghsa04140Autophagy - animal - Homo sapiens (human)
kegghsa04141Protein processing in endoplasmic reticulum - Homo sapiens (human)
kegghsa04210Apoptosis - Homo sapiens (human)
kegghsa04932Non-alcoholic fatty liver disease (NAFLD) - Homo sapiens (human)
kegghsa05010Alzheimer,s disease - Homo sapiens (human)
wikipathwaysWP2059Alzheimers Disease
wikipathwaysWP2355Corticotropin-releasing hormone signaling pathway
wikipathwaysWP2864Apoptosis-related network due to altered Notch3 in ovarian cancer
wikipathwaysWP3613Photodynamic therapy-induced unfolded protein response
wikipathwaysWP3888VEGFA-VEGFR2 Signaling Pathway

Gene-Drug: Aster Plot


Drug IDDrug NameModel Num.
iGMDRD690PRIMA-1MET3
iGMDRD60Quinoclamine3
iGMDRD356PNU-746543
iGMDRD524ISX-94
iGMDRD399Selumetinib3
iGMDRD562Navitoclax3
iGMDRD885Compound 1103
iGMDRD318PAC-13
iGMDRD294Batimastat2
iGMDRD138PX 123
iGMDRD297Austocystin D5
iGMDRD280CYTOCHALASIN B3
iGMDRD394BX-7954
iGMDRD375GW-4058333
iGMDRD427ABT7373
iGMDRD546BMS-7548076
iGMDRD781Sirolimus3
iGMDRD126Tipifarnib3
iGMDRD889Compound 7d-cis3
iGMDRD772BRD47706
iGMDRD1008SR-II-138A6
iGMDRD82Quiflapon3
iGMDRD871BRD63689
iGMDRD872BRD71376
iGMDRD329Merck603
iGMDRD289Parthenolide3
iGMDRD481Dactolisib3
iGMDRD154NSC237663
iGMDRD85Ursolic acid3
iGMDRD57Chloropentafluorobenzene3
iGMDRD560MK-22063
iGMDRD47ZM3363722
iGMDRD345Compound 10b [PMID: 11504634]3
iGMDRD523Cyanoquinoline, 113
iGMDRD512nutlin 33

Gene in drug-gene network: Network Plot

Gene-drug targets distribution

Gene Structure: PDB

Models in ERN1

​​​​