- 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 |
Citation information | RRID:IMSR_EM:06943 Research Resource Identifiers (RRID) are persistent unique ID numbers assigned to help researchers cite key resources (e.g. antibodies, model organisms and software projects) in the biomedical literature to improve transparency and reproducibility in research. See https://www.rrids.org/ for more information. |
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, Rag2tm1Fwa |
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 (EMMA strain ID EM:01783), with additional genes, Rag2 and perforin 1, 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 males, homozygous C57BL/6J females |
Breeding at archiving centre | Males are Rag2 homo, beta2m homo, HLAA2 Tg, Perf homo. Females are Rag2 homo, beta2m homo, HLAA2 Tg, Perf homo; or heterozygous for beta2m and Perf. In some cases the females are wild-type for the beta2m and Perf 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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