B6.129S(Cg)-Rag2^tm1Fwa/FwaOrl

Status	Available to order
EMMA ID	EM:00162
International strain name	B6.129S(Cg)-Rag2^tm1Fwa/FwaOrl
Alternative name	Rag2°-B6
Strain type	Targeted Mutant Strains : Knock-out
Allele/Transgene symbol	Rag2^tm1Fwa
Gene/Transgene symbol	Rag2

Information from provider

Provider	Frederick Alt
Provider affiliation	National Cancer Institute
Genetic information	Mutant mice deficient in the Rag2 gene. Obtained by mutation of the Rag2 gene by homologous recombination (insertion of the PMC1neo gene into the unique exon of Rag2 gene, leading to the deletion of 286 amino acids).
Phenotypic information	Homozygous mutants are viable even if they are smaller in size than wild-type or heterozygous littermates. Homozygous animals do not produce mature B or T lymphocytes. Deletion of a large portion of the Rag2 gene produces a total inability to initiate V(D)J rearrangement, leading to a severe combined immune deficiency (SCID).
Breeding history	129/Sv ES cells; chimaera crossed with MF1; 9 generations of backcross to C57BL/6.
References	RAG-2-deficient mice lack mature lymphocytes owing to inability to initiate V(D)J rearrangement.;Shinkai Y, Rathbun G, Lam K P, Oltz E M, Stewart V, Mendelsohn M, Charron J, Datta M, Young F, Stall A M, ;1992;Cell;68;855-67; 1547487

Information from EMMA

Archiving centre	Institut de Transgenose, INTRAGENE, Orléans, France

Disease and phenotype information

MGI allele-associated human disease models

severe combined immunodeficiency, autosomal recessive, T cell-negative, B cell-negative, Nk cell-positive / DOID:0090013

Orphanet associated rare diseases, based on orthologous gene matching

Severe combined immunodeficiency due to complete RAG1/2 deficiency / Orphanet_331206
Omenn syndrome / Orphanet_39041
Combined immunodeficiency with granulomatosis / Orphanet_157949

MGI phenotypes (allele matching)

increased granulocyte number / MGI
abnormal spleen morphology / MGI
spleen hypoplasia / MGI
decreased body size / MGI
arrested B cell differentiation / MGI
decreased IgM level / MGI
thymus hypoplasia / MGI
arrested T cell differentiation / MGI
increased susceptibility to infection / MGI
abnormal effector T cell morphology / MGI
increased natural killer cell mediated cytotoxicity / MGI
decreased double-positive T cell number / MGI
increased macrophage cell number / MGI
decreased CD4-positive, alpha beta T cell number / MGI
decreased CD8-positive, alpha-beta T cell number / MGI
lymph node hypoplasia / MGI
absent mature B cells / MGI
abnormal T cell receptor V(D)J recombination / MGI
abnormal immunoglobulin V(D)J recombination / MGI
decreased thymocyte number / MGI
lung inflammation / MGI
increased susceptibility to bacterial infection / MGI
abnormal macrophage physiology / MGI
colitis / MGI
abnormal T-helper 1 physiology / MGI
abnormal response to transplant / MGI
abnormal interferon secretion / MGI
abnormal chemokine secretion / MGI
decreased susceptibility to bacterial infection induced morbidity/mortality / MGI
abnormal intestinal mucosa morphology / MGI
abnormal B cell morphology / MGI
decreased susceptibility to parasitic infection / MGI
decreased double-negative T cell number / MGI
increased double-negative T cell number / MGI
decreased susceptibility to type IV hypersensitivity reaction / MGI
abnormal stomach mucosa morphology / MGI
abnormal intestinal epithelium morphology / MGI
abnormal B cell number / MGI
decreased pre-B cell number / MGI
decreased mature B cell number / MGI
increased immature B cell number / MGI
decreased immature B cell number / MGI
abnormal gamma-delta T cell differentiation / MGI

MGI phenotypes (gene matching)

decreased monocyte cell number / MGI
increased granulocyte number / MGI
decreased bone marrow cell number / MGI
alopecia / MGI
abnormal digestive system morphology / MGI
abnormal intestinal epithelium morphology / MGI
abnormal intestinal mucosa morphology / MGI
abnormal spleen morphology / MGI
small spleen / MGI
spleen hypoplasia / MGI
abnormal Peyer's patch morphology / MGI
abnormal thymus morphology / MGI
decreased thymocyte number / MGI
abnormal immune system cell morphology / MGI
decreased body size / MGI
abnormal humoral immune response / MGI
arrested B cell differentiation / MGI
decreased IgG level / MGI
decreased IgM level / MGI
decreased IgA level / MGI
thymus hypoplasia / MGI
arrested T cell differentiation / MGI
lung inflammation / MGI
neoplasm / MGI
abnormal B cell differentiation / MGI
no abnormal phenotype detected / MGI
small lymph nodes / MGI
abnormal lymph node morphology / MGI
abnormal lymphopoiesis / MGI
abnormal pre-B cell morphology / MGI
increased susceptibility to infection / MGI
abnormal double-negative T cell morphology / MGI
abnormal double-positive T cell morphology / MGI
increased susceptibility to bacterial infection / MGI
abnormal effector T cell morphology / MGI
abnormal macrophage physiology / MGI
abnormal B cell number / MGI
abnormal B cell physiology / MGI
abnormal immune system organ morphology / MGI
colitis / MGI
absent Peyer's patches / MGI
increased natural killer cell mediated cytotoxicity / MGI
erythroderma / MGI
abnormal lymphocyte physiology / MGI
abnormal B cell morphology / MGI
decreased lymphocyte cell number / MGI
decreased B cell number / MGI
decreased T cell number / MGI
decreased susceptibility to parasitic infection / MGI
decreased double-negative T cell number / MGI
increased double-negative T cell number / MGI
decreased double-positive T cell number / MGI
decreased T cell proliferation / MGI
cachexia / MGI
increased macrophage cell number / MGI
abnormal T-helper 1 physiology / MGI
decreased susceptibility to type IV hypersensitivity reaction / MGI
abnormal response to transplant / MGI
abnormal T cell morphology / MGI
decreased NK T cell number / MGI
decreased CD4-positive, alpha beta T cell number / MGI
decreased CD8-positive, alpha-beta T cell number / MGI
lymph node hypoplasia / MGI
decreased pre-B cell number / MGI
decreased mature B cell number / MGI
absent mature B cells / MGI
increased immature B cell number / MGI
decreased immature B cell number / MGI
abnormal gamma-delta T cell differentiation / MGI
decreased spleen white pulp amount / MGI
abnormal interferon secretion / MGI
abnormal chemokine secretion / MGI
abnormal T cell receptor V(D)J recombination / MGI
abnormal immunoglobulin V(D)J recombination / MGI
absent Hassall's corpuscle / MGI
small inguinal lymph nodes / MGI
decreased susceptibility to bacterial infection induced morbidity/mortality / MGI
increased DN3 thymocyte number / MGI
abnormal stomach mucosa morphology / MGI
small cervical lymph nodes / MGI

Literature references

RAG-2-deficient mice lack mature lymphocytes owing to inability to initiate V(D)J rearrangement.;Shinkai Y, Rathbun G, Lam K P, Oltz E M, Stewart V, Mendelsohn M, Charron J, Datta M, Young F, Stall A M, ;1992;Cell;68;855-67; 1547487
SOCS3 transactivation by PPARγ prevents IL-17-driven cancer growth.;Berger Hélène, Végran Frédérique, Chikh Madijd, Gilardi Federica, Ladoire Sylvain, Bugaut Hélène, Mignot Grégoire, Chalmin Fanny, Bruchard Mélanie, Derangère Valentin, Chevriaux Angélique, Rébé Cédric, Ryffel Bernhard, Pot Caroline, Hichami Aziz, Desvergne Béatrice, Ghiringhelli François, Apetoh Lionel, ;2013;Cancer research;73;3578-90; 23619236
Tumor-associated high endothelial venules mediate lymphocyte entry into tumors and predict response to PD-1 plus CTLA-4 combination immunotherapy.;Asrir Assia, Tardiveau Claire, Coudert Juliette, Laffont Robin, Blanchard Lucas, Bellard Elisabeth, Veerman Krystle, Bettini Sarah, Lafouresse Fanny, Vina Estefania, Tarroux Dorian, Roy Severine, Girault Isabelle, Molinaro Irma, Martins Frédéric, Scoazec Jean-Yves, Ortega Nathalie, Robert Caroline, Girard Jean-Philippe, ;2022;Cancer cell;40;318-334.e9; 35120598
Isolation and Culture of Mouse Lung ILC2s.;Schmitt Pauline, Duval Anais, Mirey Emilie, Girard Jean-Philippe, Cayrol Corinne, ;2018;Bio-protocol;8;e3032; 34532511
The Transcription Factor MAZR/PATZ1 Regulates the Development of FOXP3⁺ Regulatory T Cells.;Andersen Liisa, Gülich Alexandra Franziska, Alteneder Marlis, Preglej Teresa, Orola Maria Jonah, Dhele Narendra, Stolz Valentina, Schebesta Alexandra, Hamminger Patricia, Hladik Anastasiya, Floess Stefan, Krausgruber Thomas, Faux Thomas, Andrabi Syed Bilal Ahmad, Huehn Jochen, Knapp Sylvia, Sparwasser Tim, Bock Christoph, Laiho Asta, Elo Laura L, Rasool Omid, Lahesmaa Riitta, Sakaguchi Shinya, Ellmeier Wilfried, ;2019;Cell reports;29;4447-4459.e6; 31875552
TL1A is an epithelial alarmin that cooperates with IL-33 for initiation of allergic airway inflammation.;Schmitt Pauline, Duval Anais, Camus Mylène, Lefrançais Emma, Roga Stéphane, Dedieu Cécile, Ortega Nathalie, Bellard Elisabeth, Mirey Emilie, Mouton-Barbosa Emmanuelle, Burlet-Schiltz Odile, Gonzalez-de-Peredo Anne, Cayrol Corinne, Girard Jean-Philippe, ;2024;The Journal of experimental medicine;221;; 38597952

Information on how we integrate external resources can be found here

Order

Availabilities

Requesting frozen sperm or embryos is generally advisable wherever possible, in order to minimise the shipment of live mice.

Frozen embryos. Delivered in 4 weeks (after paperwork in place). €1740^*
Frozen sperm. Delivered in 4 weeks (after paperwork in place). €1740^*
Rederivation of mice from frozen stock, delivery time available upon request . €3880^*

Due to the dynamic nature of our processes strain availability may change at short notice. The local repository manager will advise you in these circumstances.

^* In addition users have to cover all the shipping costs (including the cost for returning dry-shippers, where applicable).

More details on pricing and delivery times

Practical information

Genotyping protocol

Example health report
(Current health report will be provided later)

Material Transfer Agreement (MTA)
For this strain no provider MTA is needed. Distribution is based on the EMMA conditions only.

EMMA conditions
Legally binding conditions for the transfer

Other EMMA strains

Not found what you were looking for? Search here for other strains available from EMMA.

B6.129S(Cg)-Rag2tm1Fwa/FwaOrl