Gene
rag2
- ID
- ZDB-GENE-990415-235
- Name
- recombination activating gene 2
- Symbol
- rag2 Nomenclature History
- Previous Names
-
- zgc:136743
- Type
- protein_coding_gene
- Location
- Chr: 25 Mapping Details/Browsers
- Description
- Enables sequence-specific DNA binding activity. Contributes to double-stranded DNA endonuclease activity. Acts upstream of or within T cell differentiation; immune system development; and protein-DNA complex assembly. Part of DNA recombinase complex and endodeoxyribonuclease complex. Is expressed in immune system and pleuroperitoneal region. Human ortholog(s) of this gene implicated in Omenn syndrome; combined cellular and humoral immune defects with granulomas; severe combined immunodeficiency; and severe combined immunodeficiency, autosomal recessive, T cell-negative, B cell-negative, Nk cell-positive. Orthologous to human RAG2 (recombination activating 2).
- Genome Resources
- Note
- None
- Comparative Information
-
- All Expression Data
- 21 figures from 18 publications
- Cross-Species Comparison
- High Throughput Data
- Thisse Expression Data
- No data available
Wild Type Expression Summary
Phenotype Summary
Mutations
| Targeting Reagent | Created Alleles | Citations |
|---|---|---|
| CRISPR1-rag2 | Ji et al., 2021 | |
| CRISPR2-rag2 | (2) | |
| CRISPR3-rag2 | (2) |
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Human Disease
| Disease Ontology Term | Multi-Species Data | OMIM Term | OMIM Phenotype ID |
|---|---|---|---|
| combined cellular and humoral immune defects with granulomas | Alliance | Combined cellular and humoral immune defects with granulomas | 233650 |
| Omenn syndrome | Alliance | Omenn syndrome | 603554 |
| severe combined immunodeficiency, autosomal recessive, T cell-negative, B cell-negative, Nk cell-positive | Alliance | Severe combined immunodeficiency, B cell-negative | 601457 |
Domain, Family, and Site Summary
| Type | InterPro ID | Name |
|---|---|---|
| Domain | IPR025162 | Recombination activating protein 2, PHD domain |
| Family | IPR004321 | V-D-J recombination activating protein 2 |
| Homologous_superfamily | IPR011011 | Zinc finger, FYVE/PHD-type |
| Homologous_superfamily | IPR011043 | Galactose oxidase/kelch, beta-propeller |
| Homologous_superfamily | IPR015915 | Kelch-type beta propeller |
Domain Details Per Protein
| Protein | Additional Resources | Length | Galactose oxidase/kelch, beta-propeller | Kelch-type beta propeller | Recombination activating protein 2, PHD domain | V-D-J recombination activating protein 2 | Zinc finger, FYVE/PHD-type |
|---|---|---|---|---|---|---|---|
| UniProtKB:Q1RLW7 | InterPro | 530 | |||||
| UniProtKB:O13034 | InterPro PDB | 530 |
Interactions and Pathways
No data available
Plasmids
No data available
| Construct | Regulatory Region | Coding Sequence | Species | Tg Lines | Citations |
|---|---|---|---|---|---|
| Tg(rag2:GFP) |
|
| 2 | (42) | |
| Tg(rag2:Hsa.MYC-ERT2) |
|
| 1 | (14) | |
| Tg(rag2:Hsa.NOTCH1-EGFP) |
|
| 1 | (6) | |
| Tg2(rag2:mCherry) |
|
| 1 | (3) | |
| Tg3(rag2:mCherry) |
|
| 1 | Leong et al., 2018 | |
| Tg4(rag2:mCherry) |
|
| 2 | (2) | |
| Tg5(rag2:mCherry) |
|
| 2 | Jiang et al., 2020 | |
| Tg(lck:Hsa.CSNK2A1_K68M,rag2:mCherry) |
|
| 1 | Zhou et al., 2020 | |
| Tg(lck:Hsa.CSNK2A1,rag2:mCherry) |
|
| 1 | Zhou et al., 2020 | |
| Tg(rag2:DsRed) |
|
| 1 | (22) |
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| Relationship | Marker Type | Marker | Accession Numbers | Citations |
|---|---|---|---|---|
| Contained in | BAC | CH211-256A21 | ZFIN Curated Data | |
| Encodes | EST | fc85e11 | ZFIN Curated Data | |
| Encodes | cDNA | MGC:136743 | ZFIN Curated Data |
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| Type | Accession # | Sequence | Length (nt/aa) | Analysis |
|---|---|---|---|---|
| RNA | RefSeq:NM_131385 (1) | 2682 nt | ||
| Genomic | GenBank:AL935203 (1) | 163572 nt | ||
| Polypeptide | UniProtKB:O13034 (1) | 530 aa |
- Cardoso, B.A., Duque, M., Gírio, A., Fragoso, R., Oliveira, M.L., Allen, J.R., Martins, L.R., Correia, N.C., Silveira, A.B., Veloso, A., Kimura, S., Demoen, L., Matthijssens, F., Jeha, S., Cheng, C., Pui, C.H., Grosso, A.R., Neto, J.L., De Almeida, S.F., Van Vlieberghe, P., Mullighan, C.G., Yunes, J.A., Langenau, D.M., Pflumio, F., Barata, J.T. (2023) CASZ1 upregulates PI3K-AKT-mTOR signaling and promotes T-cell acute lymphoblastic leukemia. Haematologica. 109(6):1713-1725
- Chen, J., Baxi, K., Lipsitt, A.E., Hensch, N.R., Wang, L., Sreenivas, P., Modi, P., Zhao, X.R., Baudin, A., Robledo, D.G., Bandyopadhyay, A., Sugalski, A., Challa, A.K., Kurmashev, D., Gilbert, A.R., Tomlinson, G.E., Houghton, P., Chen, Y., Hayes, M.N., Chen, E.Y., Libich, D.S., Ignatius, M.S. (2023) Defining function of wild-type and three patient specific TP53 mutations in a zebrafish model of embryonal rhabdomyosarcoma. eLIFE. 12:
- Rothschild, S.C., Lai, G., Tombes, R.M., Clements, W.K. (2023) Constitutively active CaMKII Drives B lineage acute lymphoblastic leukemia/lymphoma in tp53 mutant zebrafish. PLoS Genetics. 19:e1011102e1011102
- Amanda, S., Tan, T.K., Ong, J.Z.L., Theardy, M.S., Wong, R.W.J., Huang, X.Z., Ali, M.Z., Li, Y., Gong, Z., Inagaki, H., Foo, E.Y., Pang, B., Tan, S.Y., Iida, S., Sanda, T. (2022) IRF4 drives clonal evolution and lineage choice in a zebrafish model of T-cell lymphoma. Nature communications. 13:2420
- Liu, Y., Lin, Z., Peng, Y., Jiang, Y., Zhang, X., Zhu, H., Zhang, L., Chen, J., Shu, X., Luo, M., Xie, D., Chen, Y., Liao, H., Liu, M., Zhang, X., Liu, S., Wang, H., Zhou, B., Sun, H. (2022) Embryonic organizer formation disorder leads to multiorgan dysplasia in Down syndrome. Cell Death & Disease. 13:10541054
- Oliveira, M.L., Veloso, A., Garcia, E.G., Iyer, S., Pereira, C., Barreto, V.M., Langenau, D.M., Barata, J.T. (2022) Mutant IL7R collaborates with MYC to induce T-cell acute lymphoblastic leukemia. Leukemia. 36(6):1533-1540
- Gan, T., Wang, Y., Liu, Y., Schatz, D.G., Hu, J. (2021) RAG2 abolishes RAG1 aggregation to facilitate V(D)J recombination. Cell Reports. 37:109824
- He, S., Zimmerman, M.W., Layden, H.M., Berezovskaya, A., Etchin, J., Martel, M.W., Thurston, G., Jing, C.B., van Rooijen, E., Kaufman, C.K., Rodig, S.J., Zon, L.I., Patton, E.E., Mansour, M.R., Look, A.T. (2021) Synergistic melanoma cell death mediated by inhibition of both MCL1 and BCL2 in high-risk tumors driven by NF1/PTEN loss. Oncogene. 40(38):5718-5729
- Ji, J.F., Hu, C.B., Zhang, N., Huang, X., Shao, T., Fan, D.D., Lin, A.F., Xiang, L.X., Shao, J.Z. (2021) New Insights into IgZ as a Maternal Transfer Ig Contributing to the Early Defense of Fish against Pathogen Infection. Journal of immunology (Baltimore, Md. : 1950). 206(9):2001-2014
- Lin, Z., Luo, M., Zhou, B., Liu, Y., Sun, H. (2021) CFTR regulates embryonic T lymphopoiesis via Wnt signaling in zebrafish. Immunology Letters. 234:47-53
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