Gene Symbol: RAG2
Description: recombination activating 2
Alias: RAG-2, V(D)J recombination-activating protein 2, recombination activating gene 2
Species: human
Products:     RAG2

Top Publications

  1. Oettinger M, Schatz D, Gorka C, Baltimore D. RAG-1 and RAG-2, adjacent genes that synergistically activate V(D)J recombination. Science. 1990;248:1517-23 pubmed
    ..RAG-1 and RAG-2 might activate the expression of the V(D)J recombinase but, more likely, they directly participate in the recombination reaction. ..
  2. McBlane J, van Gent D, Ramsden D, Romeo C, Cuomo C, Gellert M, et al. Cleavage at a V(D)J recombination signal requires only RAG1 and RAG2 proteins and occurs in two steps. Cell. 1995;83:387-95 pubmed
    ..Here we show that purified RAG1 and RAG2 proteins are sufficient to carry out this reaction. The cleavage reaction can be divided into two distinct steps...
  3. Schwarz K, Gauss G, Ludwig L, Pannicke U, Li Z, Lindner D, et al. RAG mutations in human B cell-negative SCID. Science. 1996;274:97-9 pubmed
    ..This mutation resulted in a functional inability to form antigen receptors through genetic recombination and links a defect in one of the site-specific recombination systems to a human disease. ..
  4. Villa A, Santagata S, Bozzi F, Giliani S, Frattini A, Imberti L, et al. Partial V(D)J recombination activity leads to Omenn syndrome. Cell. 1998;93:885-96 pubmed
    ..These findings provide evidence to indicate that the immunodeficiency manifested in patients with Omenn syndrome arises from mutations that decrease the efficiency of V(D)J recombination. ..
  5. Kuo T, Schlissel M. Mechanisms controlling expression of the RAG locus during lymphocyte development. Curr Opin Immunol. 2009;21:173-8 pubmed publisher
    Recombination activating genes (RAG)1 and RAG2 are expressed in developing B and T lymphocytes and are required for the rearrangement of antigen receptor genes...
  6. Asai E, Wada T, Sakakibara Y, Toga A, Toma T, Shimizu T, et al. Analysis of mutations and recombination activity in RAG-deficient patients. Clin Immunol. 2011;138:172-7 pubmed publisher
    Mutations in the recombination activating genes (RAG1 or RAG2) can lead to a variety of immunodeficiencies...
  7. Chen Z, Xiao Y, Zhang J, Li J, Liu Y, Zhao Y, et al. Transcription factors E2A, FOXO1 and FOXP1 regulate recombination activating gene expression in cancer cells. PLoS ONE. 2011;6:e20475 pubmed publisher
    ..Recombination activating gene 1 (RAG1) and RAG2, which are essential enzymes for initiating variable-diversity-joining segment recombination, have also been found ..
  8. Laszkiewicz A, Sniezewski L, Kasztura M, Bzdzion L, Cebrat M, Kisielow P. Bidirectional activity of the NWC promoter is responsible for RAG-2 transcription in non-lymphoid cells. PLoS ONE. 2012;7:e44807 pubmed publisher
    ..We also identify evolutionarily conserved promoter fragments responsible for this bidirectional activity, and show that it is activated by transcription factor ZFP143. The possible implications of our findings are briefly discussed. ..
  9. Qiu Y, Korteweg C, Chen Z, Li J, Luo J, Huang G, et al. Immunoglobulin G expression and its colocalization with complement proteins in papillary thyroid cancer. Mod Pathol. 2012;25:36-45 pubmed publisher
    ..microdissection followed by RT-PCR, mRNA transcripts of IGHG1, Igκ, recombination activating gene 1 (RAG1), RAG2, and activation-induced cytidine deaminase genes were successfully amplified from isolated papillary thyroid cancer ..

More Information

Publications130 found, 100 shown here

  1. Liu Y, He S, Zhang Y, Xia W, Li M, Zhang C, et al. Effects of Intrauterine Growth Restriction During Late Pregnancy on the Development of the Ovine Fetal Thymus and the T-Lymphocyte Subpopulation. Am J Reprod Immunol. 2015;74:26-37 pubmed publisher
    ..In addition, there was reduced mRNA expression (P < 0.05) of T-cell receptor, apoptosis antigen 1 ligand, and RAG2 in the RG1 group...
  2. Humblet Baron S, Schonefeldt S, Garcia Perez J, Baron F, Pasciuto E, Liston A. Cytotoxic T-lymphocyte-associated protein 4-Ig effectively controls immune activation and inflammatory disease in a novel murine model of leaky severe combined immunodeficiency. J Allergy Clin Immunol. 2017;140:1394-1403.e8 pubmed publisher
    ..mutations in genes involved in the DNA recombination machinery, such as recombination-activating gene 1 (RAG1), RAG2, or DNA cross-link repair 1C (DCLRE1C)...
  3. Chow E, Quach H, Zhang Y, Wang J, Evans D, Li A, et al. Disrupted Murine Gut-to-Human Liver Signaling Alters Bile Acid Homeostasis in Humanized Mouse Liver Models. J Pharmacol Exp Ther. 2017;360:174-191 pubmed
    ..We examined these issues in FRGN [fumarylacetoacetate hydrolase (Fah-/-), recombination activating gene 2 (Rag2-/-), and interleukin 2 receptor subunit gamma (IL-2rg -/-) triple knockout] on nonobese ..
  4. Lindquist M, Zeng X, Altamura L, Daye S, Delp K, Blancett C, et al. Exploring Crimean-Congo Hemorrhagic Fever Virus-Induced Hepatic Injury Using Antibody-Mediated Type I Interferon Blockade in Mice. J Virol. 2018;92: pubmed publisher
    ..Similar experiments in CCHFV-exposed NOD-SCID-γ (NSG), Rag2-deficient, and perforin-deficient mice also demonstrated liver injury, suggesting that cytotoxic immune cells are ..
  5. Nakamura D, Edwards A, Ahn S, Thomas R, Tayade C. Compatibility of a novel thrombospondin-1 analog with fertility and pregnancy in a xenograft mouse model of endometriosis. PLoS ONE. 2015;10:e0121545 pubmed publisher
    ..These results suggest that ABT-898 effectively inhibit endometriotic lesion vascularization without affecting trans-generational pregnancy outcomes in mice. ..
  6. Diab A, Foca A, Fusil F, Lahlali T, Jalaguier P, Amirache F, et al. Polo-like-kinase 1 is a proviral host factor for hepatitis B virus replication. Hepatology. 2017;66:1750-1765 pubmed publisher
    ..In addition, a humanized liver Fah-/- /Rag2-/- /Il2rg-/- (FRG) mouse model was used to determine the antiviral effect of PLK1 inhibitor ..
  7. Albaghdadi A, Hewitt M, Putos S, Wells M, Ozolins T, Kan F. Tacrolimus in the prevention of adverse pregnancy outcomes and diabetes-associated embryopathies in obese and diabetic mice. J Transl Med. 2017;15:32 pubmed publisher
    ..o. ad libitum). HFD-BALBc-Rag2/IL2-gc female mice (n = 24) were used as HFD-immunodeficient controls...
  8. Punit S, Dubé P, Liu C, Girish N, Washington M, Polk D. Tumor Necrosis Factor Receptor 2 Restricts the Pathogenicity of CD8(+) T Cells in Mice With Colitis. Gastroenterology. 2015;149:993-1005.e2 pubmed publisher
    ..Colitis was induced in Rag2(-/-) mice by intravenous injections of naïve CD8(+) T cells isolated from C57BL/6 or Tnfr2(-/-) mice...
  9. Yazar S, Gürbüz E, Sonmez M, Cetinkaya U, Kuk S. [Investigation of potentially pathogenic free-living amoebae and their in vivo pathogenicity in water supplies of Turkey]. Mikrobiyol Bul. 2016;50:449-59 pubmed
    ..by PCR from axenic cultures were inoculated intranasally to immunocompetent and immunodeficient (athymic) [BALB/c Rag2(-/-) gamma(c)(-/-)] BALB/c mice followed by the evaluation on the 21st day by histopathological and molecular ..
  10. Fahrner R, Trochsler M, Corazza N, Graubardt N, Keogh A, Candinas D, et al. Tumor necrosis factor-related apoptosis-inducing ligand on NK cells protects from hepatic ischemia-reperfusion injury. Transplantation. 2014;97:1102-9 pubmed publisher
    ..was assessed in TRAIL-null mice and contrasted to C57BL/6J wild-type mice and after NK cell adoptive transfer in RAG2/common gamma-null mice that lack T, B, and NK cells...
  11. Ong C, Kumagai K, Brooks P, Brandenberger C, Lewandowski R, Jackson Humbles D, et al. Ozone-Induced Type 2 Immunity in Nasal Airways. Development and Lymphoid Cell Dependence in Mice. Am J Respir Cell Mol Biol. 2016;54:331-40 pubmed publisher
    ..5 parts per million (ppm) ozone for 1, 2, 4, or 9 consecutive weekdays (4 h/d). Lymphoid cell-deficient, Rag2(-/-)Il2rg(-/-) mice were similarly exposed for 9 weekdays...
  12. Larmonier C, Shehab K, Laubitz D, Jamwal D, Ghishan F, Kiela P. Transcriptional Reprogramming and Resistance to Colonic Mucosal Injury in Poly(ADP-ribose) Polymerase 1 (PARP1)-deficient Mice. J Biol Chem. 2016;291:8918-30 pubmed publisher
    ..Parp1 deficiency was evaluated in DSS-induced colitis in WT, Parp1(-/-), Rag2(-/-), and Rag2(-/-)×Parp1(-/-) double knock-out mice...
  13. Mellak S, Ait Oufella H, Esposito B, Loyer X, Poirier M, Tedder T, et al. Angiotensin II mobilizes spleen monocytes to promote the development of abdominal aortic aneurysm in Apoe-/- mice. Arterioscler Thromb Vasc Biol. 2015;35:378-88 pubmed publisher
    ..Spleen removal or lymphocyte deficiency in Apoe(-/-) Rag2(-/-) mice similarly impaired early monocyte increase in blood in response to AngII and protected against AAA ..
  14. Lee B, Lee B, Iyer V, Sleckman B, Shaffer A, Ippolito G, et al. Corrected and Republished from: BCL11A Is a Critical Component of a Transcriptional Network That Activates RAG Expression and V(D)J Recombination. Mol Cell Biol. 2018;38: pubmed publisher
    Recombination activating gene 1 (RAG1) and RAG2 are critical enzymes for initiating variable-diversity-joining [V(D)J] segment recombination, an essential process for antigen receptor expression and lymphocyte development...
  15. Lolmède K, Zakaroff Girard A, Dray C, Renoud M, Daviaud D, Burcelin R, et al. Interrelationship between lymphocytes and leptin in fat depots of obese mice revealed by changes in nutritional status. J Physiol Biochem. 2015;71:497-507 pubmed publisher
    ..obesity, the status of immune cells in subcutaneous, and epididymal fat depots in wild-type and immunodeficient Rag2-/- mice submitted to nutritional challenge, i.e., 48-h fasting and 1-week refeeding...
  16. Sato Y, Kato H, Ebina Shibuya R, Itoh Nakadai A, Okuyama R, Igarashi K. Bach2 Controls Homeostasis of Eosinophils by Restricting the Type-2 Helper Function of T Cells. Tohoku J Exp Med. 2017;241:175-182 pubmed publisher
    ..To investigate the effect of Bach2 KO CD4+ T cells upon eosinophils, we analyzed Rag2/Bach2-double deficient (dKO) mice which lack lymphocytes including CD4+ T cells...
  17. Powell N, Lo J, Biancheri P, Vossenkämper A, Pantazi E, Walker A, et al. Interleukin 6 Increases Production of Cytokines by Colonic Innate Lymphoid Cells in Mice and Patients With Chronic Intestinal Inflammation. Gastroenterology. 2015;149:456-67.e15 pubmed publisher
    ..ILCs were isolated from colons of Tbx21(-/-) × Rag2(-/-) mice (TRUC), which develop colitis; patients with inflammatory bowel disease (IBD); and patients without colon ..
  18. Carroll V, Lafferty M, Marchionni L, Bryant J, Gallo R, Garzino Demo A. Expression of HIV-1 matrix protein p17 and association with B-cell lymphoma in HIV-1 transgenic mice. Proc Natl Acad Sci U S A. 2016;113:13168-13173 pubmed
    ..the pro-B-cell stage of B-cell development, microarray analysis revealed enrichment of transcripts, including Rag1, Rag2, CD93, Vpreb1, Vpreb3, and Igll1 We confirmed RAG1 expression in Tg26 tumors, and hypothesized that HIV-1 matrix ..
  19. Ramirez J, Carvalho Costa L, Venere P, Carvalho D, Troy W, Galetti P. Testing monophyly of the freshwater fish Leporinus (Characiformes, Anostomidae) through molecular analysis. J Fish Biol. 2016;88:1204-14 pubmed publisher
    ..nuclear [myosin heavy chain 6 cardiac muscle alpha (Myh6), recombination activating gene 1 (RAG1) and recombination activating gene 2 (RAG2)] loci for 22 species of Leporinus, or c. 25% of all described species in the genus...
  20. Massart S, Redivo B, Flamion E, Mandiki S, Falisse E, Milla S, et al. The trenbolone acetate affects the immune system in rainbow trout, Oncorhynchus mykiss. Aquat Toxicol. 2015;163:109-20 pubmed publisher
    ..All combined, we conclude that trenbolone affects the rainbow trout immunity. ..
  21. Sun H. Deciphering alternative splicing and nonsense-mediated decay modulate expression in primary lymphoid tissues of birds infected with avian pathogenic E. coli (APEC). BMC Genet. 2017;18:21 pubmed publisher
    ..DETs, PSAP and STT3A, with NMD have important functions under systemic APEC infection. DETs, CDC45, CDK1, RAG2, POLR1B, PSAP, and DNASE1L3, from the same transcription start sites (TSS) indicate that cell death, cell cycle, ..
  22. Cai T, Qiu J, Ji Y, Li W, Ding Z, Suo C, et al. IL-17-producing ST2+ group 2 innate lymphoid cells play a pathogenic role in lung inflammation. J Allergy Clin Immunol. 2019;143:229-244.e9 pubmed publisher
    ..The pathogenesis of IL-17+ ILC2s was determined by transferring wild-type or Il17-/- ILC2s to Rag2-/-Il2rg-/- mice, which further induced lung inflammation...
  23. Redhead M, Portilho N, Felker A, Mohammad S, Mara D, Croy B. The Transcription Factor NFIL3 Is Essential for Normal Placental and Embryonic Development but Not for Uterine Natural Killer (UNK) Cell Differentiation in Mice. Biol Reprod. 2016;94:101 pubmed publisher
    ..We also examined implantation sites from Rag2(-/-)Il2rg(-/-) females preconditioned by adoptive transfer of Nfil3(-/-) marrow or uterine cell suspensions to ..
  24. Schmaler M, Broggi M, Rossi S. Transplantation of tail skin to study allogeneic CD4 T cell responses in mice. J Vis Exp. 2014;:e51724 pubmed publisher
    ..The absence of T cells (CD3(-/-) and Rag2(-/-) mice) allows skin graft acceptance up to 100 days, which can be overcome by transferring 2 x 10(4) wild type ..
  25. Bahjat M, Guikema J. The Complex Interplay between DNA Injury and Repair in Enzymatically Induced Mutagenesis and DNA Damage in B Lymphocytes. Int J Mol Sci. 2017;18: pubmed publisher
    ..highly ordered and regulated process that requires the recombination activating gene products 1 &amp; 2 (RAG1, RAG2)...
  26. Rodriguez Cetina Biefer H, Heinbokel T, Uehara H, Camacho V, Minami K, Nian Y, et al. Mast cells regulate CD4+ T-cell differentiation in the absence of antigen presentation. J Allergy Clin Immunol. 2018;142:1894-1908.e7 pubmed publisher
    ..C7 recombination-activating gene 2 (Rag2)-/-, and CD11b-DTR transgenic mice...
  27. Shanmugam N, Ellenbogen S, Trebicka E, Wang L, Mukhopadhyay S, Lacy Hulbert A, et al. Tumor necrosis factor α inhibits expression of the iron regulating hormone hepcidin in murine models of innate colitis. PLoS ONE. 2012;7:e38136 pubmed publisher
    ..sulfate sodium (DSS)-induced colitis in wild-type mice and the spontaneous colitis occurring in T-bet/Rag2-deficient (TRUC) mice...
  28. Wilson E, Bial J, Tarlow B, Bial G, Jensen B, Greiner D, et al. Extensive double humanization of both liver and hematopoiesis in FRGN mice. Stem Cell Res. 2014;13:404-12 pubmed publisher
    ..Immune-deficient, fumarylacetoacetate hydrolase (Fah(-/-)), Rag2(-/-) and Il2rg(-/-) deficient animals on the NOD-strain background (FRGN) were simultaneously co-transplanted with ..
  29. Saeki M, Kaminuma O, Nishimura T, Kitamura N, Mori A, Hiroi T. Th9 cells induce steroid-resistant bronchial hyperresponsiveness in mice. Allergol Int. 2017;66S:S35-S40 pubmed publisher
    ..In contrast to Th2-mediated responses, Th9-mediated airway inflammation was not affected by Dex. Th9 cells might be involved in the developmental mechanisms of steroid-resistant asthma. ..
  30. Bonnet M, Sarmento L, Martins A, Sobral D, Silva J, Demengeot J. iRAGu: A Novel Inducible and Reversible Mouse Model for Ubiquitous Recombinase Activity. Front Immunol. 2017;8:1525 pubmed publisher
    ..In this system, the RAG1 protein is constitutively expressed and functional, while the RAG2 protein, coupled to the estrogen receptor, becomes functionally active upon 4-hydroxytamoxifen (TAM) administration...
  31. Ru H, Chambers M, Fu T, Tong A, Liao M, Wu H. Molecular Mechanism of V(D)J Recombination from Synaptic RAG1-RAG2 Complex Structures. Cell. 2015;163:1138-1152 pubmed publisher
    ..The (RAG1-RAG2)2 recombinase (RAG) recognizes recombination signal sequences (RSSs) containing a heptamer, a spacer of 12 or 23 ..
  32. Moretta L, Locatelli F. Innate lymphoid cells in normal and disease: An introductory overview. Immunol Lett. 2016;179:1 pubmed publisher
    ..ILC activation is mediated by cytokines (specifically acting on different ILC groups) and/or by activating receptors that are, at least in part, the same that had been previously identified in NK cells [1]. ..
  33. Meireles C, Ribeiro A, Pinto R, Leitao C, Rodrigues P, Alves N. Thymic crosstalk restrains the pool of cortical thymic epithelial cells with progenitor properties. Eur J Immunol. 2017;47:958-969 pubmed publisher
    ..ClonoTECs wanes between the postnatal and young adult immunocompetent thymus, but it is sustained in alymphoid Rag2-/-Il2rg-/- counterparts...
  34. Sasada T, Yang Y, Lai C, Touma M, Clayton L, Liu J, et al. Disparate peptide-dependent thymic selection outcomes in beta2M-deficient mice versus TAP-1-deficient mice: implications for repertoire formation. Eur J Immunol. 2003;33:368-80 pubmed
    ..TCR-self-MHC interaction dominates Kb-based selection, subtly modulated by peptides as revealed by X-ray crystallography...
  35. Houghton J, Li H, Fan X, Liu Y, Liu J, Rao V, et al. Mutations in bone marrow-derived stromal stem cells unmask latent malignancy. Stem Cells Dev. 2010;19:1153-66 pubmed publisher
    ..To address this question, Apc(Min/+) and Apc(Min/+) Rag2(-/-) mice, which have a predilection to mammary carcinoma (as well as wild-type (wt) mice), received mesenchymal ..
  36. Sanmamed M, Rodríguez I, Schalper K, Oñate C, Azpilikueta A, Rodriguez Ruiz M, et al. Nivolumab and Urelumab Enhance Antitumor Activity of Human T Lymphocytes Engrafted in Rag2-/-IL2Rγnull Immunodeficient Mice. Cancer Res. 2015;75:3466-78 pubmed publisher
    ..Our results offer a proof of concept for the use of humanized mouse models for surrogate efficacy and histology investigations of immune checkpoint drugs and their combinations. ..
  37. Lescale C, Abramowski V, Bedora Faure M, Murigneux V, Vera G, Roth D, et al. RAG2 and XLF/Cernunnos interplay reveals a novel role for the RAG complex in DNA repair. Nat Commun. 2016;7:10529 pubmed publisher
    ..Here we show that in the context of RAG2 lacking the C-terminus domain (Rag2(c/c) mice), XLF deficiency leads to a profound lymphopenia associated with a ..
  38. Moore F, Garcia E, Lobbardi R, Jain E, Tang Q, Moore J, et al. Single-cell transcriptional analysis of normal, aberrant, and malignant hematopoiesis in zebrafish. J Exp Med. 2016;213:979-92 pubmed publisher
    ..Our platform also quantified hematopoietic defects in rag2(E450fs) mutant fish and showed that these fish have reduced T cells with a subsequent expansion of NK-lysin 4(+) ..
  39. Raveendran D, Raghavan S. Biochemical Characterization of Nonamer Binding Domain of RAG1 Reveals its Thymine Preference with Respect to Length and Position. Sci Rep. 2016;6:19091 pubmed publisher
    RAG complex consisting of RAG1 and RAG2 is a site-specific endonuclease responsible for the generation of antigen receptor diversity. It cleaves recombination signal sequence (RSS), comprising of conserved heptamer and nonamer...
  40. Carmona L, Schatz D. New insights into the evolutionary origins of the recombination-activating gene proteins and V(D)J recombination. FEBS J. 2017;284:1590-1605 pubmed publisher
    ..genes from an array of potential gene segments is mediated by the recombination-activating gene proteins RAG1 and RAG2. The RAG proteins have been proposed to originate from a transposable element (TE) as they share mechanistic and ..
  41. Rodríguez Hernández G, Hauer J, Martín Lorenzo A, Schäfer D, Bartenhagen C, García Ramírez I, et al. Infection Exposure Promotes ETV6-RUNX1 Precursor B-cell Leukemia via Impaired H3K4 Demethylases. Cancer Res. 2017;77:4365-4377 pubmed publisher
    ..KDM5C loss of function resulted in increased levels of H3K4me3, which coprecipitated with RAG2 in a human cell line model, laying the molecular basis for recombination activity...
  42. Sherrington P, Forster A, Seawright A, van Heyningen V, Rabbitts T. Human RAG2, like RAG1, is on chromosome 11 band p13 and therefore not linked to ataxia telangiectasia complementation groups. Genes Chromosomes Cancer. 1992;5:404-6 pubmed
    ..Here we report the location of a human recombination activating gene (RAG2), in addition to RAG1, on chromosome 11, band p13, thereby formally demonstrating linkage of these genes in humans ..
  43. Landree M, Wibbenmeyer J, Roth D. Mutational analysis of RAG1 and RAG2 identifies three catalytic amino acids in RAG1 critical for both cleavage steps of V(D)J recombination. Genes Dev. 1999;13:3059-69 pubmed
    RAG1 and RAG2 initiate V(D)J recombination, the process of rearranging the antigen-binding domain of immunoglobulins and T-cell receptors, by introducing site-specific double-strand breaks (DSB) in chromosomal DNA during lymphocyte ..
  44. Doherty T, Khorram N, Lund S, Mehta A, Croft M, Broide D. Lung type 2 innate lymphoid cells express cysteinyl leukotriene receptor 1, which regulates TH2 cytokine production. J Allergy Clin Immunol. 2013;132:205-13 pubmed publisher
    ..transducer and activator of transcription 6-deficient (STAT6(-/-)), and recombination-activating gene 2-deficient (RAG2(-/-)) mice by means of flow cytometry...
  45. Singh Y, Garden O, Lang F, Cobb B. MicroRNA-15b/16 Enhances the Induction of Regulatory T Cells by Regulating the Expression of Rictor and mTOR. J Immunol. 2015;195:5667-77 pubmed publisher
    ..Furthermore, the overexpression of miR-15b/16 in conventional CD4(+) T cells adoptively transferred into Rag2(-/-) mice increased the in vivo development of peripheral Tregs and diminished the severity of autoimmune colitis...
  46. Henao Tamayo M, Obregón Henao A, Arnett K, Shanley C, Podell B, Orme I, et al. Effect of bacillus Calmette-Guérin vaccination on CD4+Foxp3+ T cells during acquired immune response to Mycobacterium tuberculosis infection. J Leukoc Biol. 2016;99:605-17 pubmed publisher
    ..Transfer of Tregs into Rag2(-/-)or marked congenic mice worsened the disease course and depressed cellular influx of effector T cells into the ..
  47. Leber A, Hontecillas R, Tubau Juni N, Zoccoli Rodriguez V, Hulver M, McMillan R, et al. NLRX1 Regulates Effector and Metabolic Functions of CD4+ T Cells. J Immunol. 2017;198:2260-2268 pubmed publisher
    ..To confirm the role of NLRX1 specifically in T cells, we used an adoptive-transfer model of colitis. Rag2-/- mice receiving NLRX1-/- naive or effector T cells experienced increased disease activity ..
  48. Varenhorst A, Pritchard S, O Neal M, Hodgson E, Singh A. Determining the Effectiveness of Three-Gene Pyramids Against Aphis glycines (Hemiptera: Aphididae) Biotypes. J Econ Entomol. 2017;110:2428-2435 pubmed publisher
    ..glycines in the United States that are capable of overcoming single Rag genes, Rag1 and Rag2, as well as a two-gene pyramid of Rag1+Rag2...
  49. Ross A, Vuica M, Desiderio S. Overlapping signals for protein degradation and nuclear localization define a role for intrinsic RAG-2 nuclear uptake in dividing cells. Mol Cell Biol. 2003;23:5308-19 pubmed
    ..These results suggest that the intrinsic RAG-2 NLS functions in the nuclear uptake of RAG-2 following its reexpression in cycling cells. ..
  50. Nanni P, Nicoletti G, Palladini A, Croci S, Murgo A, Ianzano M, et al. Multiorgan metastasis of human HER-2+ breast cancer in Rag2-/-;Il2rg-/- mice and treatment with PI3K inhibitor. PLoS ONE. 2012;7:e39626 pubmed publisher
    ..We describe a model system based on a highly immunodeficient double knockout mouse, Rag2-/-;Il2rg-/-, which lacks T, B and NK cell activity...
  51. Shetty K, Schatz D. Recruitment of RAG1 and RAG2 to Chromatinized DNA during V(D)J Recombination. Mol Cell Biol. 2015;35:3701-13 pubmed publisher
    V(D)J recombination is initiated by the binding of the RAG1 and RAG2 proteins to recombination signal sequences (RSSs) that consist of conserved heptamer and nonamer sequences separated by a spacer of either 12 or 23 bp...
  52. Takeda K, Okumura K. Interferon-γ-Mediated Natural Killer Cell Activation by an Aqueous Panax ginseng Extract. Evid Based Complement Alternat Med. 2015;2015:603198 pubmed publisher
    ..or ginsenosides to wild-type (WT) C57BL/6 (B6) and BALB/c mice and to B6 mice deficient in either recombination activating gene 2 (RAG-2) or interferon-γ (IFN-γ)...
  53. Lyczek A, Arnold A, Zhang J, Campanelli J, Janowski M, Bulte J, et al. Transplanted human glial-restricted progenitors can rescue the survival of dysmyelinated mice independent of the production of mature, compact myelin. Exp Neurol. 2017;291:74-86 pubmed publisher
    ..Human or mouse glial restricted progenitors (GRPs) were transplanted into rag2-/- shiverer mouse neonates and followed for over one year...
  54. Smíd J, Carranza S, Kratochvíl L, Gvoždík V, Nasher A, Moravec J. Out of Arabia: a complex biogeographic history of multiple vicariance and dispersal events in the gecko genus Hemidactylus (Reptilia: Gekkonidae). PLoS ONE. 2013;8:e64018 pubmed publisher
    ..358 specimens sequenced for up to two mitochondrial (12S rRNA, cytochrome b) and four nuclear (mc1r, cmos, rag1, rag2) genes with 4766 bp of the concatenated alignment length...
  55. Frenzel T, Kaul M, Ernst T, Salamon J, Jäckel M, Schumacher U, et al. Magnetic resonance imaging for precise radiotherapy of small laboratory animals. Z Med Phys. 2017;27:6-12 pubmed publisher
    ..Different human cancer cells were injected at the lower trunk of pfp/rag2 and SCID mice to allow for local tumor growth...
  56. Weisz Hubshman M, Basel Vanagaite L, Krauss A, Konen O, Levy Y, Garty B, et al. Homozygous deletion of RAG1, RAG2 and 5' region TRAF6 causes severe immune suppression and atypical osteopetrosis. Clin Genet. 2017;91:902-907 pubmed publisher
    ..A biallelic microdeletion encompassing the 5' region of TRAF6, RAG1 and RAG2 genes was identified...
  57. Takahashi R, Nakatsukasa H, Shiozawa S, Yoshimura A. SOCS1 Is a Key Molecule That Prevents Regulatory T Cell Plasticity under Inflammatory Conditions. J Immunol. 2017;199:149-158 pubmed publisher
    ..When transferred into Rag2-/- mice, Tregs from Socs1fl/flLck-Cre+ mice easily lost Foxp3 ..
  58. Khan T, Iqbal A, Rahman H, Cabral Marques O, Ishfaq M, Muhammad N. Novel RAG1 mutation and the occurrence of mycobacterial and Chromobacterium violaceum infections in a case of leaky SCID. Microb Pathog. 2017;109:114-119 pubmed publisher
    ..fatal primary immunodeficiency (PID) that is caused by mutations in genes such as IL2RG, JAK3, IL7RA, RAG1, RAG2, and ADA...
  59. Pinto A, Brown N, Preham O, Doehl J, Ashwin H, Kaye P. TNF signalling drives expansion of bone marrow CD4+ T cells responsible for HSC exhaustion in experimental visceral leishmaniasis. PLoS Pathog. 2017;13:e1006465 pubmed publisher
    ..Quiescent LT-HSCs were maintained in infected RAG2 KO mice, but lost following adoptive transfer of IFN?-sufficient but not IFN?-deficient CD4+ T cells...
  60. Hayakawa S, Saito S, Nemoto N, Chishima F, Akiyama K, Shiraishi H, et al. Expression of recombinase-activating genes (RAG-1 and 2) in human decidual mononuclear cells. J Immunol. 1994;153:4934-9 pubmed
  61. Af Hällström T, Zhao H, Tian J, Rantanen V, Reese S, Nolley R, et al. A tissue graft model of DNA damage response in the normal and malignant human prostate. J Urol. 2014;191:842-9 pubmed publisher
    ..We evaluated the feasibility of maintaining tissue slice grafts at subcutaneous vs subrenal capsular sites in RAG2(-/-)γC(-/-) mice to study the DNA damage responses of normal and malignant glands...
  62. Correia A, Ferreirinha P, Botelho S, Belinha A, Leitao C, Caramalho I, et al. Predominant role of interferon-γ in the host protective effect of CD8(+) T cells against Neospora caninum infection. Sci Rep. 2015;5:14913 pubmed publisher
    ..caninum. The host protective role of IFN-γ produced by CD8(+) T cells was confirmed in N. caninum-infected RAG2-deficient mice reconstituted with CD8(+) T cells obtained from either IFN-γ-deficient or wild-type donors...
  63. Li T, Han S, Cubbage M, Zheng B. Continued expression of recombination-activating genes and TCR gene recombination in human peripheral T cells. Eur J Immunol. 2002;32:2792-9 pubmed
    ..Continued RAG gene expression, introducing and repairing of double-strand DNA breaks at the TCR loci in the periphery may have significant implications in the development of some T cell neoplasms. ..
  64. Yuki Y, Kurokawa S, Kozuka Hata H, Tokuhara D, Mejima M, Kuroda M, et al. Differential analyses of major allergen proteins in wild-type rice and rice producing a fragment of anti-rotavirus antibody. Regul Toxicol Pharmacol. 2016;76:128-36 pubmed publisher
    ..By using immunoelectron microscopy, we also showed that RAG2, a member of the α-amylase/trypsin inhibitor-like protein family, was relocated from protein bodies II to the ..
  65. Scheinert R, Haeri M, Lehmann M, Herkenham M. Therapeutic effects of stress-programmed lymphocytes transferred to chronically stressed mice. Prog Neuropsychopharmacol Biol Psychiatry. 2016;70:1-7 pubmed publisher
    ..antidepressant-like effects and elevated hippocampal cell proliferation when transferred into naïve lymphopenic Rag2(-/-) mice...
  66. Knudsen S, Clements K. World-wide species distributions in the family Kyphosidae (Teleostei: Perciformes). Mol Phylogenet Evol. 2016;101:252-266 pubmed publisher
    ..from mitochondrial markers (12s, 16s, cytb, tRNA -Pro, -Phe, -Thr and -Val) and three nuclear markers (rag1, rag2, tmo4c4)...
  67. Suzuki S, Iwamoto M, Hashimoto M, Suzuki M, Nakai M, Fuchimoto D, et al. Generation and characterization of RAG2 knockout pigs as animal model for severe combined immunodeficiency. Vet Immunol Immunopathol. 2016;178:37-49 pubmed publisher
    ..In this study, we produced RAG2-knockout pigs via somatic cell nuclear transfer and analyzed their phenotype...
  68. Dai H, Friday A, Abou Daya K, Williams A, Mortin Toth S, Nicotra M, et al. Donor SIRP? polymorphism modulates the innate immune response to allogeneic grafts. Sci Immunol. 2017;2: pubmed publisher
    ..To address this question, we studied the innate response of Rag2-/- ?c-/- mice, which lack T, B, and NK cells, to grafts from ..
  69. Lee Y, Frugoni F, Dobbs K, Tirosh I, Du L, Ververs F, et al. Characterization of T and B cell repertoire diversity in patients with RAG deficiency. Sci Immunol. 2016;1: pubmed publisher
    Recombination-activating genes 1 and 2 (RAG1 and RAG2) play a critical role in T and B cell development by initiating the recombination process that controls the expression of T cell receptor (TCR) and immunoglobulin genes...
  70. Roose J, Diehn M, Tomlinson M, Lin J, Alizadeh A, Botstein D, et al. T cell receptor-independent basal signaling via Erk and Abl kinases suppresses RAG gene expression. PLoS Biol. 2003;1:E53 pubmed
    ..Our data suggest that physiologic gene expression programs depend upon tonic activity of signaling pathways independent of receptor ligation. ..
  71. Yang J, Wang Y, Zhou T, Wong L, Tian X, Hong X, et al. Generation of Human Liver Chimeric Mice with Hepatocytes from Familial Hypercholesterolemia Induced Pluripotent Stem Cells. Stem Cell Reports. 2017;8:605-618 pubmed publisher
    ..In addition, we engrafted FH iHeps into the liver of Ldlr-/-/Rag2-/-/Il2rg-/- mice, and assessed the effect of these same medications on LDL-C clearance and ..
  72. Kalman L, Lindegren M, Kobrynski L, Vogt R, Hannon H, Howard J, et al. Mutations in genes required for T-cell development: IL7R, CD45, IL2RG, JAK3, RAG1, RAG2, ARTEMIS, and ADA and severe combined immunodeficiency: HuGE review. Genet Med. 2004;6:16-26 pubmed
    ..Mutations in any of eight known genes: IL2RG, ARTEMIS, RAG1, RAG2, ADA, CD45, JAK3, and IL7R cause SCID. Mutations in unidentified genes may also cause SCID...
  73. Matthews A, Kuo A, Ramon Maiques S, Han S, Champagne K, Ivanov D, et al. RAG2 PHD finger couples histone H3 lysine 4 trimethylation with V(D)J recombination. Nature. 2007;450:1106-10 pubmed
    ..Here we show that RAG2--an essential component of the RAG1/2 V(D)J recombinase, which mediates antigen-receptor gene assembly--contains a ..
  74. Nishihara T, Nagawa F, Imai T, Sakano H. RAG-heptamer interaction in the synaptic complex is a crucial biochemical checkpoint for the 12/23 recombination rule. J Biol Chem. 2008;283:4877-85 pubmed
    In V(D)J recombination, the RAG1 and RAG2 protein complex cleaves the recombination signal sequences (RSSs), generating a hairpin structure at the coding end...
  75. Kassmeier M, Mondal K, Palmer V, Raval P, Kumar S, Perry G, et al. VprBP binds full-length RAG1 and is required for B-cell development and V(D)J recombination fidelity. EMBO J. 2012;31:945-58 pubmed publisher
    ..These data suggest full-length RAG1 recruits a cullin RING E3 ligase complex to ubiquitylate an unknown protein(s) to limit error-prone repair during V(D)J recombination. ..
  76. Notarangelo L, Kim M, Walter J, Lee Y. Human RAG mutations: biochemistry and clinical implications. Nat Rev Immunol. 2016;16:234-46 pubmed publisher
    The recombination-activating gene 1 (RAG1) and RAG2 proteins initiate the V(D)J recombination process, which ultimately enables the generation of T cells and B cells with a diversified repertoire of antigen-specific receptors...
  77. Herrero A, Prigent J, Lombard C, Rosseels V, Daujat Chavanieu M, Breckpot K, et al. Adult-Derived Human Liver Stem/Progenitor Cells Infused 3 Days Postsurgery Improve Liver Regeneration in a Mouse Model of Extended Hepatectomy. Cell Transplant. 2017;26:351-364 pubmed publisher
    ..human liver stem/progenitor cells (ADHLSCs) at two different early time points in an immunodeficient mouse model (Rag2-/-IL2R?-/-) that had undergone a 70% hepatectomy procedure...
  78. Pol S, Polanco J, Seidman R, O Bara M, Shayya H, Dietz K, et al. Network-Based Genomic Analysis of Human Oligodendrocyte Progenitor Differentiation. Stem Cell Reports. 2017;9:710-723 pubmed publisher
    ..Following xenograft in hypomyelinating shiverer/rag2 mice, GNB4 overexpression augmented myelin synthesis and the ability of hOPCs to ensheath host axons, establishing ..
  79. Lacerda Queiroz N, Riteau N, Eastman R, Bock K, Orandle M, Moore I, et al. Mechanism of splenic cell death and host mortality in a Plasmodium yoelii malaria model. Sci Rep. 2017;7:10438 pubmed publisher
    ..These disease phenotypes were rescued in RAG2-/-, IFN-γ-/-, or T cell depleted mice, suggesting IFN-γ and T cell mediated disease ..
  80. Lavender K, Pace C, Sutter K, Messer R, Pouncey D, Cummins N, et al. An advanced BLT-humanized mouse model for extended HIV-1 cure studies. AIDS. 2017;: pubmed publisher
    ..This study aimed to: 1) Evaluate the GVHD-resistant C57BL/6?Rag2?cCD47 triple knockout (TKO)-BLT mouse as a model to establish HIV-1 latency...
  81. Bories J, Cayuela J, Loiseau P, Sigaux F. Expression of human recombination activating genes (RAG1 and RAG2) in neoplastic lymphoid cells: correlation with cell differentiation and antigen receptor expression. Blood. 1991;78:2053-61 pubmed
    ..Recently, two genes, RAG1 and RAG2, that are able to activate rearrangement of synthetic recombination substrates were cloned in mouse and a human ..
  82. Melek M, Gellert M. RAG1/2-mediated resolution of transposition intermediates: two pathways and possible consequences. Cell. 2000;101:625-33 pubmed
    During B and T cell development, the RAG1/RAG2 protein complex cleaves DNA at conserved recombination signal sequences (RSS) to initiate V(D)J recombination...
  83. Orlando L, Accomasso L, Circosta P, Turinetto V, Lantelme E, Porcedda P, et al. TCR transfer induces TCR-mediated tonic inhibition of RAG genes in human T cells. Mol Immunol. 2011;48:1369-76 pubmed publisher
    ..Lastly, we show that the TCR constructs employed in TCR gene therapy do not possess the same basal signaling transduction capability, a feature that may have therapeutic implications. ..
  84. Hasgur S, Aryee K, Shultz L, Greiner D, Brehm M. Generation of Immunodeficient Mice Bearing Human Immune Systems by the Engraftment of Hematopoietic Stem Cells. Methods Mol Biol. 2016;1438:67-78 pubmed publisher
    ..of several stocks of immunodeficient Prkdc (scid) (scid), or recombination activating 1 or 2 gene (Rag1 or Rag2) knockout mice bearing a targeted mutation in the gene encoding the IL2 receptor gamma chain (IL2rγ), has ..
  85. Ochodnicka Mackovicova K, Bahjat M, Maas C, van der Veen A, Bloedjes T, de Bruin A, et al. The DNA Damage Response Regulates RAG1/2 Expression in Pre-B Cells through ATM-FOXO1 Signaling. J Immunol. 2016;197:2918-29 pubmed publisher
    The recombination activating gene (RAG) 1 and RAG2 protein complex introduces DNA breaks at Tcr and Ig gene segments that are required for V(D)J recombination in developing lymphocytes...
  86. Wang C, Gong G, Sheh A, Muthupalani S, Bryant E, Puglisi D, et al. Interleukin-22 drives nitric oxide-dependent DNA damage and dysplasia in a murine model of colitis-associated cancer. Mucosal Immunol. 2017;10:1504-1517 pubmed publisher
    ..b>RAG2-deficient mice infected with Helicobacter hepaticus (Hh) develop colitis that progresses to lower bowel cancer...
  87. Zhou X, Gu Y, Xiao H, Kang N, Xie Y, Zhang G, et al. Combining V?9V?2 T Cells with a Lipophilic Bisphosphonate Efficiently Kills Activated Hepatic Stellate Cells. Front Immunol. 2017;8:1381 pubmed publisher
    ..The cytolytic process is largely dependent on the perforin/granzyme B pathway. In a Rag2-/-?c-/- immune-deficient mouse model, we find that V?9V?2 T cells home-in to the liver, and ..
  88. Marrella V, Poliani P, Casati A, Rucci F, Frascoli L, Gougeon M, et al. A hypomorphic R229Q Rag2 mouse mutant recapitulates human Omenn syndrome. J Clin Invest. 2007;117:1260-9 pubmed
    ..to better define the molecular and cellular pathophysiology of OS, we generated a knockin murine model carrying the Rag2 R229Q mutation previously described in several patients with OS and leaky forms of SCID...
  89. Hillion S, Dueymes M, Youinou P, Jamin C. IL-6 contributes to the expression of RAGs in human mature B cells. J Immunol. 2007;179:6790-8 pubmed
    ..In previous studies, we demonstrated that cross-linking the BCR and the CD40 induces the expression of the RAG1 and RAG2 enzymes and, thereby, secondary rearrangements...
  90. Safaei S, Pourpak Z, Moin M, Houshmand M. IL7R and RAG1/2 genes mutations/polymorphisms in patients with SCID. Iran J Allergy Asthma Immunol. 2011;10:129-32 pubmed publisher
    ..The aim of this study was on mutations detection of RAG1, RAG2, and IL7RG genes in SCID cases. Mutation detection was performed by PCR sequencing...
  91. Stone J, McMillan R, Skaar D, Bradshaw J, Jirtle R, Sikes M. DNA double-strand breaks relieve USF-mediated repression of D?2 germline transcription in developing thymocytes. J Immunol. 2012;188:2266-75 pubmed publisher
    ..Together, these data suggest a mechanism by which V(D)J recombination may feed back to regulate local D?2 recombinational accessibility during thymocyte development. ..
  92. Rodgers W, Byrum J, Sapkota H, Rahman N, Cail R, Zhao S, et al. Spatio-temporal regulation of RAG2 following genotoxic stress. DNA Repair (Amst). 2015;27:19-27 pubmed publisher
    ..antigen receptor genes occurs via the formation of DNA double strand breaks (DSBs) through the activity of RAG1 and RAG2. The co-existence of RAG-independent DNA DSBs generated by genotoxic stressors potentially increases the risk of ..