B6.Cg-Ptprc^a Rag2^tm1Fwa Foxp3^tm1.1Mal Tg(TcraH-Y,TcrbH-Y)1Pas/Orl

Status	Available to order
EMMA ID	EM:02419
International strain name	B6.Cg-Ptprc^a Rag2^tm1Fwa Foxp3^tm1.1Mal Tg(TcraH-Y,TcrbH-Y)1Pas/Orl
Alternative name	Simone
Strain type	Transgenic Strains
Allele/Transgene symbol	Tg(TcraH-Y, TcrbH-Y)1Pas, Foxp3.1^tm1Mal, Ptprc^a
Gene/Transgene symbol	Tg(TcraH-Y,TcrbH-Y)1Pas, Foxp3, Ptprc, Rag2

Information from provider

Provider	David GROSS
Provider affiliation	INSERM U580
Additional owner	O. Lantz (Marilyn) and B. Malissen (Foxp3 EGFP)
Genetic information	TCR transgenic T cells with "green" regulatory T cells (Tregs). The Foxp3 IRES-EGFP targeted knock-in allele (Foxp3 EGFP) was put on the Marilyn background. Transgenic for T(TCR)Valpha 1.1/Vbeta6 receptor specific of the H-Y male antigen presented by I-a b. The recognized peptide (NAGFNSNRANSSRSS) comes from Dby. As the clone originates from a F1 H-2b/k mouse, the TCR does not react with H-2k or H-2b. The mice of this strain are also homozygous for a Rag2 knock-out allele and Ptprc^a (CD45.1). An IRES-EGFP cassette was introduced in the 3'UTR region of FoxP3 gene, exon 11. The autodeleter neomycin-cre recombinase cassette was used.
Phenotypic information	None.
References	Gamma chain required for naïve CD4+ T cell survival but not for antigen proliferation.;Lantz O, Grandjean I, Matzinger P, Di Santo J P, ;2000;Nature immunology;1;54-8; 10881175 Th2 lymphoproliferative disorder of LatY136F mutant mice unfolds independently of TCR-MHC engagement and is insensitive to the action of Foxp3+ regulatory T cells.;Wang Ying, Kissenpfennig Adrien, Mingueneau Michael, Richelme Sylvie, Perrin Pierre, Chevrier Stéphane, Genton Céline, Lucas Bruno, DiSanto James P, Acha-Orbea Hans, Malissen Bernard, Malissen Marie, ;2008;Journal of immunology (Baltimore, Md. : 1950);180;1565-75; 18209052

Information from EMMA

Archiving centre	Institut de Transgenose, INTRAGENE, Orléans, France

Disease and phenotype information

Orphanet associated rare diseases, based on orthologous gene matching

Immune dysregulation-polyendocrinopathy-enteropathy-X-linked syndrome / Orphanet_37042
T-B+ severe combined immunodeficiency due to CD45 deficiency / Orphanet_169157
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)

enlarged spleen / MGI
abnormal Peyer's patch morphology / MGI
decreased thymocyte number / MGI
scaly skin / MGI
dermatitis / MGI
weight loss / MGI
thymus hypoplasia / MGI
conjunctivitis / MGI
liver inflammation / MGI
lung inflammation / MGI
abnormal T cell differentiation / MGI
no abnormal phenotype detected / MGI
abnormal dendritic cell physiology / MGI
abnormal gut-associated lymphoid tissue morphology / MGI
no phenotypic analysis / MGI
lymph node inflammation / MGI
abnormal regulatory T cell morphology / MGI
increased regulatory T cell number / MGI
decreased regulatory T cell number / MGI
increased T cell number / MGI
decreased T cell number / MGI
decreased double-positive T cell number / MGI
cachexia / MGI
bradykinesia / MGI
abnormal mesenteric lymph node morphology / MGI
increased susceptibility to autoimmune disorder / MGI
immune system phenotype / MGI
abnormal CD4-positive, alpha-beta T cell physiology / MGI
increased susceptibility to type IV hypersensitivity reaction / MGI
increased T cell apoptosis / MGI
increased dendritic cell number / MGI
abnormal intraepithelial T cell morphology / MGI
increased sensitivity to induced morbidity/mortality / MGI
mortality/aging / MGI
abnormal long bone metaphysis morphology / MGI
abnormal leukocyte cell number / MGI
increased neutrophil cell number / MGI
decreased leukocyte cell number / MGI
extramedullary hematopoiesis / MGI
abnormal erythropoiesis / MGI
enlarged spleen / MGI
spleen hyperplasia / MGI
enlarged lymph nodes / MGI
decreased thymocyte number / MGI
abnormal immune system cell morphology / MGI
abnormal myelination / MGI
abnormal oligodendrocyte morphology / MGI
abnormal osteoclast physiology / MGI
abnormal digestion / MGI
abnormal immune system physiology / MGI
abnormal humoral immune response / MGI
thymus hypoplasia / MGI
arrested T cell differentiation / MGI
abnormal T cell activation / MGI
liver inflammation / MGI
lung inflammation / MGI
premature death / MGI
abnormal T cell differentiation / MGI
no abnormal phenotype detected / MGI
abnormal bone marrow cell morphology/development / MGI
abnormal lymphopoiesis / MGI
abnormal double-positive T cell morphology / MGI
increased susceptibility to viral infection / MGI
abnormal T cell physiology / MGI
abnormal B cell number / MGI
increased IgA level / MGI
abnormal immune system organ morphology / MGI
glomerulonephritis / MGI
increased urine protein level / MGI
abnormal cytokine secretion / MGI
no phenotypic analysis / MGI
kidney failure / MGI
oliguria / MGI
abnormal thymocyte activation / MGI
abnormal T cell subpopulation ratio / MGI
increased anti-double stranded DNA antibody level / MGI
abnormal T cell selection / MGI
abnormal positive T cell selection / MGI
increased B-1 B cell number / MGI
abnormal osteoclast morphology / MGI
increased B cell number / MGI
increased T cell number / MGI
decreased B cell number / MGI
decreased T cell number / MGI
abnormal response to infection / MGI
diarrhea / MGI
decreased cytotoxic T cell cytolysis / MGI
increased double-negative T cell number / MGI
decreased double-positive T cell number / MGI
decreased B cell proliferation / MGI
decreased T cell proliferation / MGI
increased susceptibility to autoimmune disorder / MGI
immune system phenotype / MGI
hematopoietic system phenotype / MGI
decreased susceptibility to type I hypersensitivity reaction / MGI
CNS inflammation / MGI
increased T cell apoptosis / MGI
abnormal T cell morphology / MGI
increased NK cell number / MGI
abnormal memory T cell number / MGI
increased memory T cell number / MGI
decreased memory T cell number / MGI
absent T cells / MGI
decreased CD4-positive, alpha beta T cell number / MGI
abnormal CD4-positive T cell differentiation / MGI
decreased CD8-positive, alpha-beta T cell number / MGI
abnormal CD8-positive, alpha-beta T cell differentiation / MGI
increased single-positive T cell number / MGI
decreased single-positive T cell number / MGI
increased plasma cell number / MGI
lymph node hyperplasia / MGI
abnormal granulocyte differentiation / MGI
increased dendritic cell number / MGI
decreased B-1a cell number / MGI
increased B-1b cell number / MGI
decreased B-1b cell number / MGI
decreased follicular B cell number / MGI
increased transitional stage B cell number / MGI
abnormal follicular B cell physiology / MGI
decreased B-2 B cell number / MGI
decreased mature B cell number / MGI
abnormal B cell activation / MGI
abnormal leukocyte morphology / MGI
decreased gamma-delta intraepithelial T cell number / MGI
abnormal CD4-positive, alpha-beta intraepithelial T cell morphology / MGI
increased spleen red pulp amount / MGI
increased spleen white pulp amount / MGI
increased IgG2a level / MGI
increased plasmacytoid dendritic cell number / MGI
abnormal chemokine secretion / MGI
decreased memory B cell number / MGI
decreased mast cell degranulation / MGI
abnormal intraepithelial T cell morphology / MGI
abnormal intraepithelial T cell number / MGI
decreased oligodendrocyte number / MGI
increased DN3 thymocyte number / MGI
decreased DN4 thymocyte number / MGI
abnormal NK cell physiology / MGI
mortality/aging / MGI
decreased CD4-positive, alpha-beta memory T cell number / MGI
decreased CD8-positive, alpha-beta memory T cell number / MGI
lethality at weaning, incomplete penetrance / MGI
increased alpha-beta T cell number / MGI
decreased CD8-positive, naive alpha-beta T cell number / MGI
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

Gamma chain required for naïve CD4+ T cell survival but not for antigen proliferation.;Lantz O, Grandjean I, Matzinger P, Di Santo J P, ;2000;Nature immunology;1;54-8; 10881175
Th2 lymphoproliferative disorder of LatY136F mutant mice unfolds independently of TCR-MHC engagement and is insensitive to the action of Foxp3+ regulatory T cells.;Wang Ying, Kissenpfennig Adrien, Mingueneau Michael, Richelme Sylvie, Perrin Pierre, Chevrier Stéphane, Genton Céline, Lucas Bruno, DiSanto James P, Acha-Orbea Hans, Malissen Bernard, Malissen Marie, ;2008;Journal of immunology (Baltimore, Md. : 1950);180;1565-75; 18209052

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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^*
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

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B6.Cg-Ptprca Rag2tm1Fwa Foxp3tm1.1Mal Tg(TcraH-Y,TcrbH-Y)1Pas/Orl