ERN1 - endoplasmic reticulum to nucleus signaling 1 Gene

Also Known as IRE1; IRE1P; IRE1a; hIRE1p

Species: Homo sapiens

Gene Type: protein coding

Gene ID: 2081

About ERN1

Cytogenetic location: 17q23.3 Genomic coordinates (GRCh38): 17:64,039,142-64,130,144 (from NCBI)

This gene has 10 transcripts (splice variants), 133 orthologues and 1 paralogue. Ubiquitous expression in adrenal (RPKM 14.0), pancreas (RPKM 8.7) and 24 other tissues.

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]

ERN1 Products (3)

mRNA	Protein	Name
XM_047435591.1	XP_047291547.1	serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1
NM_152461.2
NM_001433.5	NP_001424.3	serine/threonine-protein kinase/endoribonuclease IRE1 precursor

Gene Ontology

Molecular Function
Biological Process
Cellular Component

Molecular Function GO Annotation	Evidence	References	Source
enables ADP binding	IDA IDA: Inferred from direct assay	21317875	GOA
enables ATP binding	IDA IDA: Inferred from direct assay	9637683	GOA
enables Hsp70 protein binding	IPI IPI: Inferred from physical interaction	20625543	GOA
enables Hsp90 protein binding	IDA IDA: Inferred from direct assay	12446770	GOA
enables RNA endonuclease activity	IDA IDA: Inferred from direct assay	9637683	GOA
enables enzyme binding	IPI IPI: Inferred from physical interaction	23103912	GOA
enables identical protein binding	IPI IPI: Inferred from physical interaction	12637535	GOA
enables magnesium ion binding	IDA IDA: Inferred from direct assay	9637683	GOA
enables platelet-derived growth factor receptor binding	IPI IPI: Inferred from physical interaction	26315405	GOA
enables protein binding	IPI IPI: Inferred from physical interaction	11278723	GOA
enables protein homodimerization activity	IDA IDA: Inferred from direct assay	29198525	GOA
enables protein homodimerization activity	IPI IPI: Inferred from physical interaction	21317875	GOA
enables protein serine/threonine kinase activity	IDA IDA: Inferred from direct assay	9637683	GOA

Biological Process GO Annotation	Evidence	References	Source
involved in IRE1-mediated unfolded protein response	IDA IDA: Inferred from direct assay	9637683	GOA
involved in IRE1-mediated unfolded protein response	IMP IMP: Inferred from mutant phenotype	28128204	GOA
involved in cellular response to glucose stimulus	IDA IDA: Inferred from direct assay	20103773	GOA
involved in cellular response to unfolded protein	IDA IDA: Inferred from direct assay	19328063	GOA
involved in cellular response to unfolded protein	IMP IMP: Inferred from mutant phenotype	20625543	GOA
involved in cellular response to vascular endothelial growth factor stimulus	IDA IDA: Inferred from direct assay	23529610	GOA
involved in endothelial cell proliferation	IDA IDA: Inferred from direct assay	23529610	GOA
involved in insulin metabolic process	IDA IDA: Inferred from direct assay	20103773	GOA
involved in mRNA splicing, via endonucleolytic cleavage and ligation	IDA IDA: Inferred from direct assay	11779464	GOA
involved in mRNA splicing, via endonucleolytic cleavage and ligation	IMP IMP: Inferred from mutant phenotype	20625543	GOA
involved in peptidyl-serine autophosphorylation	IDA IDA: Inferred from direct assay	20103773	GOA
involved in peptidyl-serine trans-autophosphorylation	IMP IMP: Inferred from mutant phenotype	21317875	GOA
involved in positive regulation of JUN kinase activity	IDA IDA: Inferred from direct assay	10650002	GOA
involved in positive regulation of RNA splicing	IDA IDA: Inferred from direct assay	11779464	GOA
involved in positive regulation of endoplasmic reticulum unfolded protein response	IMP IMP: Inferred from mutant phenotype	28128204	GOA
involved in positive regulation of vascular associated smooth muscle cell proliferation	IMP IMP: Inferred from mutant phenotype	26315405	GOA
involved in protein autophosphorylation	IDA IDA: Inferred from direct assay	9637683	GOA
involved in protein phosphorylation	IDA IDA: Inferred from direct assay	9637683	GOA
involved in response to endoplasmic reticulum stress	IDA IDA: Inferred from direct assay	10650002	GOA

Cellular Component GO Annotation	Evidence	References	Source
part of IRE1-RACK1-PP2A complex	IDA IDA: Inferred from direct assay	20103773	GOA
part of IRE1-TRAF2-ASK1 complex	IDA IDA: Inferred from direct assay	23000344	GOA
located in cytoplasm	IDA IDA: Inferred from direct assay	19622636	GOA
located in endoplasmic reticulum	IDA IDA: Inferred from direct assay	23529610	GOA
located in endoplasmic reticulum membrane	IDA IDA: Inferred from direct assay	9637683	GOA

EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern

ERN1 Protein Structure

Pkinase

Ribonuc_2-5A

0
200
400
600
800
977 a.a.

Protein Preferred Names	Protein Names
serine/threonine-protein kinase/endoribonuclease IRE1	ER to nucleus signalling 1 inositol-requiring 1 inositol-requiring enzyme 1 inositol-requiring protein 1 ire1-alpha protein kinase/endoribonuclease

ERN1 Protein-protein interaction Information

Type	Protein Name	Protein ID	Interactor	Interactor Species	Interactor ID	Detection Method	References
Intra	ERN1	O75460	TAOK3	Homo sapiens	Q9H2K8	Y2H	11278723
Intra	ERN1	O75460	TAOK3	Homo sapiens	Q9H2K8	Anti Tag CoIP	11278723
Intra	ERN1	O75460	YIPF5	Homo sapiens	Q969M3	Anti Bait CoIP	25742138
Intra	ERN1	O75460	YIPF5	Homo sapiens	Q969M3	Confocal	25742138
Intra	ERN1	O75460	HSPA5	Homo sapiens	P11021	Anti Bait CoIP	27238082
Intra	ERN1	O75460	TRAF2	Homo sapiens	Q12933	Anti Tag CoIP	11278723
Intra	ERN1	O75460	TRAF2	Homo sapiens	Q12933	Anti Bait CoIP	11278723
Intra	ERN1	O75460	ERN1	Homo sapiens	O75460	Comig Non-Denat Gel	12637535
Intra	ERN1	O75460	BAX	Homo sapiens	Q07812	Pull Down	16645094
Intra	ERN1	O75460	ERN1	Homo sapiens	O75460	X-Ray Diffraction	21317875

Cross: Cross-species interaction Intra: Intraspecies interaction

Recombinant ERN1 Proteins

Cat. No.	Product Name	Accession	Purity
HY-P73256	IRE1 Protein, Human (Active, sf9, His-GST)	O75460-1 (P465-L977)	≥ 90%, as determined by reducing SDS-PAGE.
HY-P73858	IRE1 Protein, Human (sf9)	O75460-1 (P465-L977)	≥ 80%, as determined by reducing SDS-PAGE.

ERN1 Antibodies

Cat. No.	Product Name	Application	Reactivity
HY-P80828	Phospho-IRE1 (Ser724) Antibody (YA182)	WB, IP	Human
HY-P80828A	Phospho-IRE1 (Ser724) Antibody (YA182)(PBS only)	WB, IP	Human
HY-P83860	IRE1 Antibody (YA3557)	WB, IHC-P, ELISA	Human
HY-P83860A	IRE1 Antibody (YA3557)(PBS only)	WB, IHC-P, ELISA	Human
HY-P86330	IRE1 alpha Antibody (YA6022)	WB, IHC-P, ICC/IF, IP, ELISA	Human, Mouse, Rat

Related Diseases

Diseases	Alias
Diabetes Mellitus	Diabetes
Diabetic Encephalopathy
Nervous System Disease	Abnormality Of The Nervous System Nervous System Diseases Nervous System Disorder
Amyotrophic Lateral Sclerosis 1	Amyotrophic Lateral Sclerosis ALS Lou Gehrig Disease Amyotrophic Lateral Sclerosis Type 1 Charcot Disease ALS1 Amyotrophic Lateral Sclerosis, Susceptibility To Fals Lou Gehrig'S Disease Mnd Motor Neuron Disease Familial Amyotrophic Lateral Sclerosis Amyotrophic Lateral Sclerosis 1, Familial Amyotrophic Lateral Sclerosis 1, Autosomal Dominant Motor Neuron Disease, Bulbar Motor Neurone Disease Amyotrophic Lateral Sclerosis With Dementia Dementia With Amyotrophic Lateral Sclerosis Motor Neuron Disease, Amyotrophic Lateral Sclerosis Sclerosis, Lateral, Amyotrophic Sclerosis, Lateral, Amyotrophic, Type 1 Amyotrophic Sclerosis Als - [Amyotrophic Lateral Sclerosis] Wasting Palsy Amyotrophic Paralysis Amyotrophy Lateral Sclerosis Wasting Paralysis Spinal Progressive Amyotrophy Progressive Atrophic Paralysis
Retinitis Pigmentosa	RP Rod-Cone Dystrophy Autosomal Recessive Retinitis Pigmentosa Non-Syndromic Retinitis Pigmentosa Pericentral Pigmentary Retinopathy Pigmentary Retinopathy Tapetoretinal Degeneration Rcd Retinitis Pigmentosa Autosomal Recessive ARRP Retinitis Pigmentosa, Autosomal Recessive Retinitis Pigmentosa 1
Wolfram Syndrome	Didmoad Syndrome Didmoad Diabetes Insipidus And Mellitus With Optic Atrophy And Deafness Wfs Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy, And Deafness Didmoadud Diabetes Insipidus-Diabetes Mellitus-Optic Atrophy-Deafness Syndrome Diabetes Insipidus-Diabetes Mellitus-Optic Atrophy-Hearing Loss Syndrome
Cystic Fibrosis	Mucoviscidosis CF Pseudomonas Aeruginosa, Susceptibility To Chronic Infection By, In Cystic Fibrosis Pseudomonas Aeruginosa Chronic Infection By, In Cystic Fibrosis Cystic Fibrosis Lung Disease, Modifier Of Cystic Fibrosis Of Pancreas Fibrocystic Disease Of Pancreas Cf - [Cystic Fibrosis] Cystic Fibrosis Nos Fibrocystic Disease Fibrocystic Disease Of The Pancreas Mucoviscidosis Of Pancreas Nonproliferative Fibrocystic Disease Pancreatic Cystic Fibrosis
Type 2 Diabetes Mellitus	Insulin Resistance NIDDM Diabetes Mellitus, Non-Insulin-Dependent Type 2 Diabetes T2D Noninsulin-Dependent Diabetes Mellitus Diabetes Mellitus, Type Ii Maturity-Onset Diabetes Insulin Resistance, Severe, Digenic Diabetes Mellitus, Type 2 Diabetes Mellitus, Noninsulin-Dependent Diabetes Mellitus, Noninsulin-Dependent, Association With Diabetes Mellitus, Noninsulin-Dependent, Late Onset Hypertension, Insulin Resistance-Related, Susceptibility To Insulin Resistance, Susceptibility To Non-Insulin-Dependent Diabetes Mellitus Type Ii Diabetes Mellitus Adult-Onset Diabetes Mellitus Maturity-Onset Diabetes Mellitus Diabetes Mellitus Type 2 Type Ii Diabetes Type 2 Diabetes Mellitus, Susceptibility To Diabetes, Type 2 Diabetes Mellitus, Noninsulin-Dependent, Susceptibility To Diabetes Mellitus, Non-Insulin-Dependent, Susceptibility To Diabetes Mellitus, Type 2, Susceptibility To Diabetes Mellitus, Noninsulin-Dependent, 2 Diabetes Mellitus, Type Ii, Susceptibility To Hypertension, Insulin Resistance-Related Adult-Onset Diabetes Aodm Diabetes Mellitus, Adult-Onset Diabetes Mellitus Type Ii Diabetes Mellitus Type 2, Susceptibility To Diabetes, Type Ii, Susceptibility To Diabetes Type 2 Diabetes Mellitus Adult Onset Diabetes Maturity Onset Diabetes Nonketotic Diabetes Non-Insulin Dependent Diabetes Mellitus T2dm - [Type 2 Diabetes Mellitus] Niddm - [Non Insulin Dependent Diabetes Mellitus] Dm2 Dm Type Ii Diabetic Type 2 Insulin Requiring Type 2 Diabetes Noninsulin Dependent Diabetes Non-Insulin-Dependent Diabetes Mellitus Without Complications Diabetes Due To Insulin Secretory Defect Diabetes Mellitus Due To Insulin Secretory Defect Non-Insulin-Dependent Diabetes Of The Young Senile Diabetes Nonketotic Hyperglycaemia Stable Diabetes
Epiphyseal Dysplasia, Multiple, With Early-Onset Diabetes Mellitus	Wolcott-Rallison Syndrome Med-Iddm Syndrome Iddm-Med Syndrome Wolcott Rallison Syndrome WRS Epiphyseal Dysplasia Multiple With Early-Onset Diabetes Mellitus Early-Onset Diabetes Mellitus With Multiple Epiphyseal Dysplasia Multiple Epiphyseal Dysplasia With Early-Onset Diabetes Mellitus
Ovarian Cancer	Ovarian Carcinoma Ovarian Neoplasm Malignant Tumour Of Ovary Epithelial Ovarian Cancer Neoplasm Of Ovary Ovarian Neoplasms Ovarian Cancers Malignant Neoplasm Of Ovary Primary Malignant Neoplasm Of Ovary Ovarian Cancer, Somatic Malignant Ovarian Tumor Ovary Neoplasm Primary Ovarian Cancer Tumor Of The Ovary Cancer Of The Ovary Malignant Neoplasm Of The Ovary Malignant Tumor Of The Ovary Ovarian Malignant Tumor OC Ovarian Carcinomas Cancer, Ovarian Cancer Of Ovary Ovary Cancer Ca Ovary

Diseases	Alias
Waldenstroem'S Macroglobulinemia	Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma

Related Agents

Pre-clinical Phase

Pre-clinical Phase

Bioactive Molecules (5)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-174497	eGFP mRNA		90%	Unkown
HY-136735	IRE1α kinase-IN-1	Inhibitor	98.94%	Unkown
HY-RS04509	Ern1 Rat Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-RS04507	ERN1 Human Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-RS04508	Ern1 Mouse Pre-designed siRNA Set A	Pre-designed Set	/	Unkown

Orthologs Information

Species	Symbol	Source	ID
Macaca mulatta	ERN1	VGNC	VGNC:72429
Bos taurus	ERN1	VGNC	VGNC:58373
Canis familiaris	ERN1	VGNC	VGNC:40464
Rattus norvegicus	ERN1	RGD	RGD:1559716
Felis catus	ERN1	VGNC	VGNC:61952
Mus musculus	ERN1	MGD	MGI:1930134
Others	ERN1	NCBI