- preweaning lethality, incomplete penetrance / IMPC
- preweaning lethality, complete penetrance / IMPC
- small superior vagus ganglion / IMPC
- embryonic lethality prior to tooth bud stage / IMPC
- abnormal skin morphology / IMPC
- hydrometra / IMPC
- increased circulating phosphate level / IMPC
- decreased mean corpuscular hemoglobin concentration / IMPC
STOCK Rag2tm1Fwa B2mtm1Unc Prf1tm1Sdz H2-Ab1b-tm1Doi Tg(HLA-A/H2-D/B2M)1Bpe Tg(HLA-DRA*0101,HLA-DRB1*0101)1Dma/Orl
Status | Available to order |
EMMA ID | EM:06943 |
International strain name | STOCK Rag2tm1Fwa B2mtm1Unc Prf1tm1Sdz H2-Ab1b-tm1Doi Tg(HLA-A/H2-D/B2M)1Bpe Tg(HLA-DRA*0101,HLA-DRB1*0101)1Dma/Orl |
Alternative name | SURE-L1/Rag2°/° Prf°/° |
Strain type | Targeted Mutant Strains : Knock-out |
Allele/Transgene symbol | Prf1tm1Sdz, B2mtm1Unc, H2-Ab1b-tm1Doi, Tg(HLA-A/H2-D/B2M)1Bpe, Tg(HLA-DRA*0101, HLA-DRB1*0101)1Dma |
Gene/Transgene symbol | Prf1, B2m, H2-Ab1, Tg(HLA-A/H2-D/B2M)1Bpe, Tg(HLA-DRA*0101,HLA-DRB1*0101)1Dma, Rag2 |
Information from provider
Provider | Yu Chun LONE |
Provider affiliation | INSERM U1014 |
Genetic information | This line is a combination of the previously cryopreserved line, Sure-L1 (EM:01783), with additional genes, Rag2 and perforin knocked out. The literature describes two comparable immunodeficient models that may be used for transplant recipient studies, Rag2-/- GammaC-/- and Rag2-/- Prf-/-. The first requires very early transfer of stem cells in newborns to overcome ganglion atrophy that is associated with this model. Rag2-/- GammaC-/- mice also show increased transplant acceptance frequency, as compared to Rag2-/- Prf-/- mice. However, for ease of use and taking advantage of their normal ganglion structures, Rag2-/- Prf-/- provide a good model for transplantation studies, as stem cells can be transferred in adult. |
Phenotypic information | Only checked by total genotyping and partial phenotyping for the absence of both T and B cells. |
Breeding history | We transferred mice in heterozygous status to CDTA-EMMA Orleans, where we continue breeding until obtaining homozygous mice. |
References |
|
Homozygous fertile | yes |
Homozygous viable | yes |
Homozygous matings required | yes |
Immunocompromised | yes |
Information from EMMA
Archiving centre | Institut de Transgenose, INTRAGENE, Orléans, France |
Animals used for archiving | homozygous C57BL/6J, homozygous C57BL/6J |
Breeding at archiving centre | Males are Rag2, B2m (beta2m), Prf1 (Perf) homozygous knock-out and Tg(HLA-DRA*0101,HLA-DRB1*0101)1Dma (HLAA2) transgenic. Females are Rag2, B2m, Prf1 homozygous knock-out and Tg(HLA-DRA*0101,HLA-DRB1*0101)1Dma (HLAA2) transgenic, or B2m and Prf1 heterozygous knock-out. In some cases females are wild-type for the B2m and Prf1 genes. |
Stage of embryos | 2-cell |
Disease and phenotype information
Orphanet associated rare diseases, based on orthologous gene matching
- Fatal post-viral neurodegenerative disorder / Orphanet_391343
- Familial hemophagocytic lymphohistiocytosis / Orphanet_540
- Immunodeficiency by defective expression of MHC class I / Orphanet_34592
- Variant ABeta2M amyloidosis / Orphanet_314652
- Severe combined immunodeficiency due to complete RAG1/2 deficiency / Orphanet_331206
- Omenn syndrome / Orphanet_39041
- Combined immunodeficiency with granulomatosis / Orphanet_157949
IMPC phenotypes (gene matching)
MGI phenotypes (allele matching)
- decreased level of surface class I molecules / MGI
- decreased CD8-positive, alpha-beta T cell number / MGI
- decreased IgG level / MGI
- abnormal pancreas morphology / MGI
- decreased susceptibility to bacterial infection / MGI
- increased IgM level / MGI
- decreased susceptibility to autoimmune diabetes / MGI
- decreased circulating serum albumin level / MGI
- increased T-helper 1 cell number / MGI
- abnormal interleukin level / MGI
- abnormal tumor necrosis factor level / MGI
- increased susceptibility to infection induced morbidity/mortality / MGI
- absent CD8-positive, alpha-beta T cells / MGI
- abnormal pancreatic beta cell morphology / MGI
- abnormal T cell number / MGI
- insulitis / MGI
- periinsulitis / MGI
- increased incidence of induced tumors / MGI
- impaired natural killer cell mediated cytotoxicity / MGI
- decreased leukocyte cell number / MGI
- decreased neutrophil cell number / MGI
- decreased bone marrow cell number / MGI
- ruffled hair / MGI
- demyelination / MGI
- weight loss / MGI
- hypoactivity / MGI
- hunched posture / MGI
- increased circulating triglyceride level / MGI
- abnormal body temperature homeostasis / MGI
- abnormal T cell activation / MGI
- brain inflammation / MGI
- liver inflammation / MGI
- abnormal lymph node morphology / MGI
- abnormal spleen white pulp morphology / MGI
- increased susceptibility to viral infection / MGI
- decreased erythrocyte cell number / MGI
- thrombocytopenia / MGI
- lymph node inflammation / MGI
- abnormal lymphocyte physiology / MGI
- abnormal behavior / MGI
- abnormal cytotoxic T cell physiology / MGI
- decreased cytotoxic T cell cytolysis / MGI
- pancytopenia / MGI
- lethargy / MGI
- increased circulating aspartate transaminase level / MGI
- behavior/neurological phenotype / MGI
- immune system phenotype / MGI
- decreased body temperature / MGI
- CNS inflammation / MGI
- increased spleen red pulp amount / MGI
- increased circulating tumor necrosis factor level / MGI
- decreased interferon-gamma secretion / MGI
- increased circulating interferon-alpha level / MGI
- increased circulating interferon-gamma level / MGI
- increased circulating interleukin-10 level / MGI
- increased circulating interleukin-6 level / MGI
- increased circulating interleukin-18 level / MGI
- increased circulating interleukin-1 beta level / MGI
- decreased NK cell degranulation / MGI
- abnormal brain meninges morphology / MGI
- increased susceptibility to viral infection induced morbidity/mortality / MGI
- abnormal circulating cytokine level / MGI
- abnormal circulating fibrinogen level / MGI
- abnormal macrophage activation involved in immune response / MGI
- increased circulating lactate dehydrogenase level / MGI
- abnormal inflammatory response / MGI
- increased B cell derived lymphoma incidence / MGI
- premature death / MGI
- increased susceptibility to infection / MGI
- increased susceptibility to bacterial infection / MGI
- abnormal leukocyte physiology / MGI
- abnormal female reproductive system physiology / MGI
- increased susceptibility to parasitic infection / MGI
- increased interleukin-10 secretion / MGI
- increased sensitivity to induced morbidity/mortality / MGI
- decreased tumor latency / MGI
- abnormal NK cell physiology / MGI
- increased lymphoma incidence / MGI
- abnormal circulating glucose level / MGI
- decreased pancreatic beta cell number / MGI
- increased physiological sensitivity to xenobiotic / MGI
- increased T cell derived lymphoma incidence / MGI
- increased lung adenocarcinoma incidence / MGI
- increased sarcoma incidence / MGI
- abnormal tumor morphology / MGI
- increased tumor latency / MGI
- 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
- 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
- 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
- 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)
- absent CD8-positive, alpha-beta T cells / MGI
- decreased IgG level / MGI
- decreased level of surface class I molecules / MGI
- abnormal pancreas morphology / MGI
- decreased susceptibility to bacterial infection / MGI
- abnormal CD8-positive, alpha-beta cytotoxic T cell morphology / MGI
- increased IgM level / MGI
- insulitis / MGI
- abnormal lateral geniculate nucleus morphology / MGI
- decreased susceptibility to autoimmune diabetes / MGI
- decreased susceptibility to parasitic infection / MGI
- abnormal cytotoxic T cell physiology / MGI
- abnormal pancreatic beta cell morphology / MGI
- decreased circulating serum albumin level / MGI
- hemochromatosis / MGI
- abnormal T cell number / MGI
- decreased CD8-positive, alpha-beta T cell number / MGI
- increased T-helper 1 cell number / MGI
- increased gamma-delta T cell number / MGI
- increased gamma-delta intraepithelial T cell number / MGI
- increased tumor necrosis factor secretion / MGI
- increased interleukin-3 secretion / MGI
- decreased interleukin-4 secretion / MGI
- abnormal interleukin level / MGI
- abnormal tumor necrosis factor level / MGI
- increased susceptibility to infection induced morbidity/mortality / MGI
- abnormal immune system morphology / MGI
- abnormal spleen morphology / MGI
- abnormal immune system cell morphology / MGI
- hemolytic anemia / MGI
- postnatal growth retardation / MGI
- abnormal immune system physiology / MGI
- abnormal humoral immune response / MGI
- decreased IgG level / MGI
- decreased IgM level / MGI
- autoimmune response / MGI
- reduced fertility / MGI
- premature death / MGI
- abnormal T cell differentiation / MGI
- no abnormal phenotype detected / MGI
- abnormal spleen periarteriolar lymphoid sheath morphology / MGI
- abnormal spleen marginal zone morphology / MGI
- abnormal dendritic cell physiology / MGI
- abnormal CD4-positive, alpha beta T cell morphology / MGI
- abnormal T cell physiology / MGI
- abnormal dendritic cell antigen presentation / MGI
- abnormal B cell physiology / MGI
- abnormal immunoglobulin level / MGI
- increased IgM level / MGI
- abnormal immune system organ morphology / MGI
- increased urine protein level / MGI
- abnormal cytokine secretion / MGI
- abnormal artery development / MGI
- abnormal response/metabolism to endogenous compounds / MGI
- absent CD4-positive, alpha beta T cells / MGI
- abnormal lymphocyte physiology / MGI
- insulitis / MGI
- increased anti-double stranded DNA antibody level / MGI
- increased anti-single stranded DNA antibody level / MGI
- increased anti-erythrocyte antigen antibody level / MGI
- decreased susceptibility to experimental autoimmune encephalomyelitis / MGI
- decreased susceptibility to experimental autoimmune myasthenia gravis / MGI
- decreased regulatory T cell number / MGI
- increased B cell number / MGI
- decreased susceptibility to parasitic infection / MGI
- increased susceptibility to parasitic infection / MGI
- abnormal level of surface class II molecules / MGI
- increased double-negative T cell number / MGI
- decreased susceptibility to autoimmune disorder / MGI
- cardiovascular system phenotype / MGI
- immune system phenotype / MGI
- abnormal CD4-positive, alpha-beta T cell physiology / MGI
- abnormal response to transplant / MGI
- increased CD4-positive, alpha beta T cell number / MGI
- decreased CD4-positive, alpha beta T cell number / MGI
- increased CD8-positive, alpha-beta T cell number / MGI
- decreased CD8-positive, alpha-beta T cell number / MGI
- decreased single-positive T cell number / MGI
- abnormal spleen B cell follicle morphology / MGI
- absent spleen germinal center / MGI
- decreased IgG1 level / MGI
- decreased tumor necrosis factor secretion / MGI
- decreased interleukin-12 secretion / MGI
- abnormal lymph node cell ratio / MGI
- abnormal neuron proliferation / MGI
- abnormal circulating glucose level / MGI
- decreased leukocyte cell number / MGI
- decreased neutrophil cell number / MGI
- decreased bone marrow cell number / MGI
- ruffled hair / MGI
- demyelination / MGI
- weight loss / MGI
- hypoactivity / MGI
- hunched posture / MGI
- increased circulating triglyceride level / MGI
- abnormal body temperature homeostasis / MGI
- abnormal T cell activation / MGI
- increased activated T cell number / MGI
- abnormal inflammatory response / MGI
- brain inflammation / MGI
- liver inflammation / MGI
- increased incidence of induced tumors / MGI
- increased B cell derived lymphoma incidence / MGI
- increased T cell derived lymphoma incidence / MGI
- increased lung adenocarcinoma incidence / MGI
- increased sarcoma incidence / MGI
- premature death / MGI
- no abnormal phenotype detected / MGI
- abnormal lymph node morphology / MGI
- abnormal spleen white pulp morphology / MGI
- increased susceptibility to infection / MGI
- decreased susceptibility to bacterial infection / MGI
- increased susceptibility to bacterial infection / MGI
- increased susceptibility to viral infection / MGI
- abnormal leukocyte physiology / MGI
- decreased erythrocyte cell number / MGI
- decreased circulating alanine transaminase level / MGI
- abnormal cytokine secretion / MGI
- thrombocytopenia / MGI
- decreased pancreatic beta cell number / MGI
- abnormal tumor morphology / MGI
- abnormal female reproductive system physiology / MGI
- increased tumor growth/size / MGI
- lymph node inflammation / MGI
- abnormal lymphocyte physiology / MGI
- insulitis / MGI
- decreased susceptibility to autoimmune diabetes / MGI
- abnormal behavior / MGI
- increased susceptibility to parasitic infection / MGI
- impaired natural killer cell mediated cytotoxicity / MGI
- abnormal cytotoxic T cell physiology / MGI
- decreased cytotoxic T cell cytolysis / MGI
- pancytopenia / MGI
- lethargy / MGI
- increased circulating aspartate transaminase level / MGI
- behavior/neurological phenotype / MGI
- immune system phenotype / MGI
- decreased body temperature / MGI
- periinsulitis / MGI
- CNS inflammation / MGI
- decreased T cell apoptosis / MGI
- increased spleen red pulp amount / MGI
- increased circulating tumor necrosis factor level / MGI
- increased tumor necrosis factor secretion / MGI
- increased interferon-gamma secretion / MGI
- decreased interferon-gamma secretion / MGI
- increased circulating interferon-alpha level / MGI
- increased circulating interferon-gamma level / MGI
- increased circulating interleukin-10 level / MGI
- increased circulating interleukin-6 level / MGI
- increased circulating interleukin-18 level / MGI
- increased circulating interleukin-1 beta level / MGI
- increased interleukin-10 secretion / MGI
- decreased NK cell degranulation / MGI
- increased physiological sensitivity to xenobiotic / MGI
- abnormal brain meninges morphology / MGI
- increased sensitivity to induced morbidity/mortality / MGI
- increased susceptibility to viral infection induced morbidity/mortality / MGI
- increased tumor latency / MGI
- abnormal circulating cytokine level / MGI
- abnormal circulating fibrinogen level / MGI
- decreased tumor latency / MGI
- abnormal NK cell physiology / MGI
- abnormal macrophage activation involved in immune response / MGI
- increased circulating lactate dehydrogenase level / MGI
- increased lymphoma incidence / 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
- A mouse model of human adaptive immune functions: HLA-A2.1-/HLA-DR1-transgenic H-2 class I-/class II-knockout mice.;Pajot Anthony, Michel Marie-Louise, Fazilleau Nicolas, Pancré Véronique, Auriault Claude, Ojcius David M, Lemonnier François A, Lone Yu-Chun, ;2004;European journal of immunology;34;3060-9; 15468058
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