STOCK Rag2^tm1Fwa B2m^tm1Unc Prf1^tm1Sdz H2-Ab1^b-tm1Doi Il2rg^tm1Sug Tg(HLA-A/H2-D/B2M)1Bpe Tg(HLA-DRA0101,HLA-DRB10101)1Dma/Orl

Status	Available to order
EMMA ID	EM:06327
Citation information	RRID:IMSR_EM:06327 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 Rag2^tm1Fwa B2m^tm1Unc Prf1^tm1Sdz H2-Ab1^b-tm1Doi Il2rg^tm1Sug Tg(HLA-A/H2-D/B2M)1Bpe Tg(HLA-DRA0101,HLA-DRB10101)1Dma/Orl
Alternative name	SURE-L1/Rag2°/°GammaC°/°Prf°/°
Strain type	Targeted Mutant Strains : Knock-out
Allele/Transgene symbol	Prf1^tm1Sdz, B2m^tm1Unc, H2-Ab1^b-tm1Doi, Rag2^tm1Fwa, Il2rg^tm1Sug, Tg(HLA-A/H2-D/B2M)1Bpe, Tg(HLA-DRA0101,HLA-DRB10101)1Dma
Gene/Transgene symbol	Prf1, B2m, H2-Ab1, Rag2, Il2rg, Tg(HLA-A/H2-D/B2M)1Bpe, Tg(HLA-DRA0101,HLA-DRB10101)1Dma

Information from provider

Provider	Yu Chun LONE
Provider affiliation	INSERM U1014
Genetic information	The Sure-L1 (EMMA strain ID EM:01783) strain was additionally knocked-out in the Rag2, Il2rg (GammaC) and perforin 1 genes.
Phenotypic information	HLA-A2+/+; DR1+/+; IAbeta°/°; Rag2°/°; GammaC°/°; Prf°/°
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
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: transgenic for Tg(HLA-A/H2-D/B2M)1Bpe (HLA-A2) and Tg(HLA-DRA0101,HLA-DRB10101)1Dma (DR1); homozygous knock-out for H2-Ab1 (IAbeta), Rag2, perforin 1 and Il2rg (GammaC); heterozygous knock-out/wild-type for B2m (Beta2m). Females: transgenic for Tg(HLA-A/H2-D/B2M)1Bpe (HLA-A2) and Tg(HLA-DRA0101,HLA-DRB10101)1Dma (DR1); homozygous knock-out for H2-Ab1 (IAbeta), Rag2, perforin 1 and Il2rg (GammaC); heterozygous knock-out/wild-type for B2m (Beta2m).
Stage of embryos	2-cell

Disease and phenotype information

Orphanet associated rare diseases, based on orthologous gene matching

Omenn syndrome / Orphanet_39041
T-B+ severe combined immunodeficiency due to gamma chain deficiency / Orphanet_276
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
Combined immunodeficiency with granulomatosis / Orphanet_157949

IMPC phenotypes (gene matching)

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

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
abnormal estrous cycle / MGI
abnormal maternal decidual layer morphology / MGI
reproductive system phenotype / MGI
absent uterine NK cells / MGI
prolonged estrous cycle / MGI
prolonged diestrus / MGI
prolonged metestrus / MGI
abnormal placenta metrial gland morphology / MGI
abnormal decidua basalis morphology / 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
spleen hypoplasia / MGI
decreased thymocyte number / MGI
decreased IgM level / MGI
thymus hypoplasia / MGI
abnormal estrous cycle / MGI
abnormal dendritic cell physiology / MGI
abnormal hematopoietic system morphology/development / MGI
decreased IgE level / MGI
absent Peyer's patches / MGI
abnormal thymocyte activation / MGI
abnormal maternal decidual layer morphology / MGI
abnormal class switch recombination / MGI
decreased T cell number / MGI
impaired natural killer cell mediated cytotoxicity / MGI
decreased T cell proliferation / MGI
reproductive system phenotype / MGI
absent NK cells / MGI
absent uterine NK cells / MGI
decreased plasma cell number / MGI
decreased myeloid dendritic cell number / MGI
decreased pre-B cell number / MGI
decreased mature B cell number / MGI
abnormal leukocyte morphology / MGI
abnormal alpha-beta intraepithelial T cell morphology / MGI
absent spleen germinal center / MGI
decreased spleen white pulp amount / MGI
abnormal splenic cell ratio / MGI
prolonged estrous cycle / MGI
prolonged diestrus / MGI
prolonged metestrus / MGI
abnormal placenta metrial gland morphology / MGI
abnormal decidua basalis morphology / 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

Information on how we integrate external resources can be found here

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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

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

Material Transfer Agreement (MTA)
Distribution of this strain is subject to a provider MTA. Both signing of the MTA and submission of the online EMMA Mutant Request Form are required before material can be shipped.

EMMA conditions
Legally binding conditions for the transfer

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STOCK Rag2tm1Fwa B2mtm1Unc Prf1tm1Sdz H2-Ab1b-tm1Doi Il2rgtm1Sug Tg(HLA-A/H2-D/B2M)1Bpe Tg(HLA-DRA*0101,HLA-DRB1*0101)1Dma/Orl