Gene
ern2
- ID
- ZDB-GENE-090312-15
- Name
- endoplasmic reticulum to nucleus signaling 2
- Symbol
- ern2 Nomenclature History
- Previous Names
-
- si:ch211-14k19.11
- Type
- protein_coding_gene
- Location
- Chr: 1 Mapping Details/Browsers
- Description
- Predicted to enable RNA endonuclease activity; protein serine/threonine kinase activity; and unfolded protein binding activity. Predicted to be involved in IRE1-mediated unfolded protein response and intrinsic apoptotic signaling pathway in response to endoplasmic reticulum stress. Predicted to act upstream of or within endoplasmic reticulum unfolded protein response; mRNA processing; and protein phosphorylation. Predicted to be located in membrane. Predicted to be part of IRE1-TRAF2-ASK1 complex. Is expressed in liver and male organism. Human ortholog(s) of this gene implicated in hepatocellular carcinoma. Orthologous to several human genes including ERN2 (endoplasmic reticulum to nucleus signaling 2).
- Genome Resources
- Note
- None
- Comparative Information
-
- All Expression Data
- 8 figures from 7 publications
- Cross-Species Comparison
- High Throughput Data
- Thisse Expression Data
- No data available
Wild Type Expression Summary
- All Phenotype Data
- No data available
- Cross-Species Comparison
- Alliance
Phenotype Summary
Mutations
| Allele | Type | Localization | Consequence | Mutagen | Supplier |
|---|---|---|---|---|---|
| sa12750 | Allele with one point mutation | Unknown | Premature Stop | ENU | |
| sa17230 | Allele with one point mutation | Unknown | Splice Site | ENU | |
| sa18038 | Allele with one point mutation | Unknown | Premature Stop | ENU | |
| sa25525 | Allele with one point mutation | Unknown | Splice Site | ENU | |
| sa39549 | Allele with one point mutation | Unknown | Premature Stop | ENU |
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No data available
Human Disease
Domain, Family, and Site Summary
| Type | InterPro ID | Name |
|---|---|---|
| Active_site | IPR008271 | Serine/threonine-protein kinase, active site |
| Domain | IPR000719 | Protein kinase domain |
| Domain | IPR010513 | KEN domain |
| Family | IPR045133 | Serine/threonine-protein kinase/endoribonuclease IRE1/2-like |
| Homologous_superfamily | IPR011009 | Protein kinase-like domain superfamily |
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Domain Details Per Protein
| Protein | Additional Resources | Length | KEN domain | KEN domain superfamily | Protein kinase domain | Protein kinase-like domain superfamily | Pyrrolo-quinoline quinone beta-propeller repeat | Quinoprotein alcohol dehydrogenase-like superfamily | Serine/threonine-protein kinase, active site | Serine/threonine-protein kinase/endoribonuclease IRE1/2-like | WD40/YVTN repeat-like-containing domain superfamily |
|---|---|---|---|---|---|---|---|---|---|---|---|
| UniProtKB:A0A8M3B496 | InterPro | 776 | |||||||||
| UniProtKB:A0A8M9Q0A4 | InterPro | 777 | |||||||||
| UniProtKB:A0A8M1QQI8 | InterPro | 930 | |||||||||
| UniProtKB:A0A8M9PPK0 | InterPro | 931 |
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Interactions and Pathways
No data available
Plasmids
No data available
No data available
| Relationship | Marker Type | Marker | Accession Numbers | Citations |
|---|---|---|---|---|
| Contained in | BAC | CH211-14K19 | ZFIN Curated Data |
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| Type | Accession # | Sequence | Length (nt/aa) | Analysis |
|---|---|---|---|---|
| RNA | RefSeq:XM_001919315 (1) | 3137 nt | ||
| Genomic | GenBank:BX004779 (1) | 196036 nt | ||
| Polypeptide | UniProtKB:A0A8M9PPK0 (1) | 931 aa |
- Doll, L., Welte, K., Skokowa, J., Bajoghli, B. (2024) A JAGN1-associated severe congenital neutropenia zebrafish model revealed an altered G-CSFR signaling and UPR activation. Blood advances. 8(15):4050-4065
- Huttner, I.G., Santiago, C.F., Jacoby, A., Cheng, D., Trivedi, G., Cull, S., Cvetkovska, J., Chand, R., Berger, J., Currie, P.D., Smith, K.A., Fatkin, D. (2023) Loss of Sec-1 Family Domain-Containing 1 (scfd1) Causes Severe Cardiac Defects and Endoplasmic Reticulum Stress in Zebrafish. Journal of cardiovascular development and disease. 10(10):
- Crouzier, L., Richard, E.M., Diez, C., Alzaeem, H., Denus, M., Cubedo, N., Delaunay, T., Glendenning, E., Baxendale, S., Liévens, J.C., Whitfield, T.T., Maurice, T., Delprat, B. (2022) Morphological, behavioral and cellular analyses revealed different phenotypes in Wolfram syndrome wfs1a and wfs1b zebrafish mutant lines. Human molecular genetics. 31(16):2711-2727
- Crouzier, L., Richard, E.M., Diez, C., Denus, M., Peyrel, A., Alzaeem, H., Cubedo, N., Delaunay, T., Maurice, T., Delprat, B. (2022) NCS1 overexpression restored mitochondrial activity and behavioral alterations in a zebrafish model of Wolfram syndrome. Molecular therapy. Methods & clinical development. 27:295-308
- Dai, W., Wang, K., Zhen, X., Huang, Z., Liu, L. (2022) Magnesium isoglycyrrhizinate attenuates acute alcohol-induced hepatic steatosis in a zebrafish model by regulating lipid metabolism and ER stress. Nutrition & metabolism. 19:23
- Di Paola, D., Natale, S., Iaria, C., Cordaro, M., Crupi, R., Siracusa, R., D'Amico, R., Fusco, R., Impellizzeri, D., Cuzzocrea, S., Spanò, N., Gugliandolo, E., Peritore, A.F. (2022) Intestinal Disorder in Zebrafish Larvae (Danio rerio): The Protective Action of N-Palmitoylethanolamide-oxazoline. Life (Basel, Switzerland). 12(1):
- Lasbleiz, C., Peyrel, A., Tarot, P., Sarniguet, J., Crouzier, L., Cubedo, N., Delprat, B., Rossel, M., Maurice, T., Liévens, J.C. (2022) Sigma-1 receptor agonist PRE-084 confers protection against TAR DNA-binding protein-43 toxicity through NRF2 signalling. Redox Biology. 58:102542102542
- Crouzier, L., Denus, M., Richard, E.M., Tavernier, A., Diez, C., Cubedo, N., Maurice, T., Delprat, B. (2021) Sigma-1 Receptor Is Critical for Mitochondrial Activity and Unfolded Protein Response in Larval Zebrafish. International Journal of Molecular Sciences. 22(20):
- Singh, M.K., Jayarajan, R., Varshney, S., Upadrasta, S., Singh, A., Yadav, R., Scaria, V., Sengupta, S., Shanmugam, D., Shalimar, ., Sivasubbu, S., Gandotra, S., Sachidanandan, C. (2021) Chronic systemic exposure to IL6 leads to deregulation of glycolysis and fat accumulation in the zebrafish liver. Biochimica et biophysica acta. Molecular and cell biology of lipids. 1866(5):158905
- Zhang, Y., Jiao, Y., Tao, Y., Li, Z., Yu, H., Han, S., Yang, Y. (2021) Monobutyl phthalate can induce autophagy and metabolic disorders by activating the ire1a-xbp1 pathway in zebrafish liver. Journal of hazardous materials. 412:125243
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