NOD.129-Rag1^tm1Bal B2m^tm1Unc H2-Ab1^b-tm1Doi/Orl

Status	Available to order
EMMA ID	EM:01638
International strain name	NOD.129-Rag1^tm1Bal B2m^tm1Unc H2-Ab1^b-tm1Doi/Orl
Alternative name	NOD-IAbeta-beta2m-RAG1-/-
Strain type	Targeted Mutant Strains : Knock-out
Allele/Transgene symbol	H2-Ab1^b-tm1Doi, B2m^tm1Unc, Rag1^tm1Bal
Gene/Transgene symbol	H2-Ab1, B2m, Rag1

Information from provider

Provider	Francoise Lepault
Provider affiliation	INSERM U561 - Hopital Saint Vincent de Paul
Genetic information	NOD mice deficient for IAbeta (H2-Ab1), beta-2 microglobulin (B2m) and Rag1 molecules.
Phenotypic information	These mice have no T, B, NKT lymphocytes and no MHC molecules. Do not develop diabetes. Breeding more difficult than with wild-type mice.
Breeding history	NOD mice deficient for IAbeta were crossed with NOD mice deficient for beta2m to generate NOD mice deficient for both molecules. The latter mice were then crossed with NOD mice deficient for Rag1. The three NOD knock-out strains were used after 10 backcrosses to the NOD background.
References	Mice lacking MHC class II molecules.;Cosgrove D, Gray D, Dierich A, Kaufman J, Lemeur M, Benoist C, Mathis D, ;1991;Cell;66;1051-66; 1909605 Major histocompatibility complex class I molecules are required for the development of insulitis in non-obese diabetic mice.;Katz J, Benoist C, Mathis D, ;1993;European journal of immunology;23;3358-60; 8258349 RAG-1 and RAG-2, adjacent genes that synergistically activate V(D)J recombination.;Oettinger M A, Schatz D G, Gorka C, Baltimore D, ;1990;Science (New York, N.Y.);248;1517-23; 2360047

Information from EMMA

Archiving centre	Institut de Transgenose, INTRAGENE, Orléans, France

Disease and phenotype information

Orphanet associated rare diseases, based on orthologous gene matching

Severe combined immunodeficiency due to complete RAG1/2 deficiency / Orphanet_331206
Combined immunodeficiency due to partial RAG1 deficiency / Orphanet_231154
Omenn syndrome / Orphanet_39041
Combined immunodeficiency with granulomatosis / Orphanet_157949
Immunodeficiency by defective expression of MHC class I / Orphanet_34592
Variant ABeta2M amyloidosis / Orphanet_314652

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

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

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
decreased neutrophil cell number / MGI
increased granulocyte number / MGI
spleen hypoplasia / MGI
small thymus / MGI
absent lymphocyte / MGI
scaly skin / MGI
dermatitis / MGI
thick skin / MGI
epidermal hyperplasia / MGI
mixed cellular infiltration to dermis / MGI
abnormal retina morphology / MGI
arrested B cell differentiation / MGI
decreased IgG level / MGI
decreased IgM level / MGI
thymus hypoplasia / MGI
arrested T cell differentiation / MGI
autoimmune response / MGI
ear inflammation / MGI
decreased inflammatory response / MGI
abnormal lymphatic vessel morphology / MGI
abnormal coat/hair pigmentation / MGI
abnormal eye morphology / MGI
abnormal skin pigmentation / MGI
abnormal respiratory system morphology / MGI
abnormal B cell differentiation / MGI
abnormal T cell differentiation / MGI
abnormal inguinal lymph node morphology / MGI
abnormal lymphopoiesis / MGI
abnormal double-negative T cell morphology / MGI
abnormal double-positive T cell morphology / MGI
decreased immunoglobulin level / MGI
abnormal immunoglobulin level / MGI
abnormal bone remodeling / MGI
decreased vascular permeability / MGI
nervous system phenotype / MGI
spontaneous chromosome breakage / MGI
abnormal class switch recombination / MGI
increased eosinophil cell number / MGI
decreased lymphocyte cell number / MGI
decreased B cell number / MGI
decreased T cell number / MGI
abnormal response to infection / MGI
abnormal level of surface class II molecules / MGI
impaired natural killer cell mediated cytotoxicity / MGI
increased double-negative T cell number / MGI
decreased double-positive T cell number / MGI
behavior/neurological phenotype / MGI
skeleton phenotype / MGI
increased macrophage cell number / MGI
absent late pro-B cells / MGI
abnormal T cell morphology / MGI
increased NK cell number / MGI
absent T cells / MGI
absent B cells / MGI
decreased CD4-positive, alpha beta T cell number / MGI
decreased CD8-positive, alpha-beta T cell number / MGI
increased pro-B cell number / MGI
decreased mature B cell number / MGI
absent mature B cells / MGI
absent immature B cells / MGI
increased immature B cell number / MGI
decreased gamma-delta T cell number / MGI
decreased susceptibility to induced colitis / MGI
abnormal T cell receptor V(D)J recombination / MGI
abnormal immunoglobulin V(D)J recombination / MGI
abnormal T cell receptor beta chain V(D)J recombination / MGI
abnormal immunoglobulin heavy chain V(D)J recombination / MGI
abnormal thymus epithelium morphology / MGI
abnormal neuron proliferation / MGI

Literature references

Mice lacking MHC class II molecules.;Cosgrove D, Gray D, Dierich A, Kaufman J, Lemeur M, Benoist C, Mathis D, ;1991;Cell;66;1051-66; 1909605
Major histocompatibility complex class I molecules are required for the development of insulitis in non-obese diabetic mice.;Katz J, Benoist C, Mathis D, ;1993;European journal of immunology;23;3358-60; 8258349
RAG-1 and RAG-2, adjacent genes that synergistically activate V(D)J recombination.;Oettinger M A, Schatz D G, Gorka C, Baltimore D, ;1990;Science (New York, N.Y.);248;1517-23; 2360047

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
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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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NOD.129-Rag1tm1Bal B2mtm1Unc H2-Ab1b-tm1Doi/Orl