MGI phenotypes (allele matching)
B6.Cg-Nfkbiatm1.1Kbp/Ieg
| Status | Available to order |
| EMMA ID | EM:06270 |
| Citation information | RRID:IMSR_EM:06270 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 | B6.Cg-Nfkbiatm1.1Kbp/Ieg |
| Alternative name | Ikba del |
| Strain type | Targeted Mutant Strains : Knock-out |
| Allele/Transgene symbol | Nfkbiatm1.1Kbp |
| Gene/Transgene symbol | Nfkbia |
Information from provider
| Provider | Rudolf Rupec |
| Provider affiliation | Derma AG |
| Genetic information | Deletion of the promoter region containing essential regulatory NF-kappaB binding sites, the core promoter, and the first two exons. |
| Phenotypic information | Homozygous KO mice show severe growth retardation at the age of 3 days and onward, death after 7 days. Hypergranulopoiesis, myeloproliferative disease and upregulation of Notch1 in neutrophils and Jagged1 in non-hematopoietic cells. |
| Breeding history | Ikba+/Delta mice were backcrossed for more than seven generations with C57BL/6-Ly5.2 mice. |
| References |
|
| Homozygous fertile | no |
| Homozygous viable | no |
| Homozygous matings required | no |
| Immunocompromised | not known |
Information from EMMA
| Archiving centre | Helmholtz Zentrum Muenchen - German Research Center for Environmental Health (GmbH), Oberschleißheim, Germany |
Disease and phenotype information
Orphanet associated rare diseases, based on orthologous gene matching
- Nasopharyngeal carcinoma / Orphanet_150
- Hypohidrotic ectodermal dysplasia with immunodeficiency / Orphanet_98813
MGI phenotypes (gene matching)
- increased leukocyte cell number / MGI
- increased monocyte cell number / MGI
- extramedullary hematopoiesis / MGI
- abnormal erythropoiesis / MGI
- increased bone marrow cell number / MGI
- increased granulocyte number / MGI
- small spleen / MGI
- increased thymocyte number / MGI
- decreased thymocyte number / MGI
- reddish skin / MGI
- abnormal skin condition / MGI
- scaly skin / MGI
- dermatitis / MGI
- flaky skin / MGI
- spontaneous skin ulceration / MGI
- abnormal epidermis stratum basale morphology / MGI
- abnormal epidermis stratum granulosum morphology / MGI
- abnormal epidermis stratum corneum morphology / MGI
- hyperkeratosis / MGI
- decreased body weight / MGI
- decreased body size / MGI
- anemia / MGI
- abnormal myelopoiesis / MGI
- postnatal growth retardation / MGI
- abnormal humoral immune response / MGI
- decreased IgA level / MGI
- abnormal T cell activation / MGI
- increased activated T cell number / MGI
- autoimmune response / MGI
- acanthosis / MGI
- premature death / MGI
- abnormal B cell differentiation / MGI
- no abnormal phenotype detected / MGI
- abnormal granulocyte morphology / MGI
- abnormal macrophage physiology / MGI
- increased IgG level / MGI
- increased IgA level / MGI
- increased IgE level / MGI
- chronic inflammation / MGI
- abnormal keratinocyte morphology / MGI
- increased anti-insulin autoantibody level / MGI
- increased pre-B cell number / MGI
- spleen atrophy / MGI
- thymus atrophy / MGI
- dry skin / MGI
- increased anti-nuclear antigen antibody level / MGI
- decreased regulatory T cell number / MGI
- decreased B cell number / MGI
- abnormal cell differentiation / MGI
- decreased double-negative T cell number / MGI
- increased double-positive T cell number / MGI
- decreased double-positive T cell number / MGI
- decreased B cell proliferation / MGI
- decreased T cell proliferation / MGI
- increased B cell proliferation / MGI
- increased T cell proliferation / MGI
- increased macrophage cell number / MGI
- abnormal cell physiology / MGI
- abnormal T cell number / MGI
- increased T cell apoptosis / MGI
- increased memory T cell number / MGI
- decreased CD4-positive, alpha beta T cell number / MGI
- decreased CD8-positive, alpha-beta T cell number / MGI
- increased single-positive T cell number / MGI
- abnormal granulocyte differentiation / MGI
- decreased Peyer's patch number / MGI
- small Peyer's patches / MGI
- decreased follicular B cell number / MGI
- abnormal marginal zone B cell morphology / MGI
- decreased marginal zone B cell number / MGI
- increased pro-B cell number / MGI
- decreased B-2 B cell number / MGI
- decreased mature B cell number / MGI
- increased immature B cell number / MGI
- absent spleen germinal center / MGI
- decreased IgG1 level / MGI
- decreased IgG2b level / MGI
- increased circulating tumor necrosis factor level / MGI
- increased circulating interleukin-17 level / MGI
- increased circulating interleukin-1 alpha level / MGI
- abnormal tumor necrosis factor level / MGI
- increased susceptibility to bacterial infection induced morbidity/mortality / MGI
- decreased transitional stage T2 B cell number / MGI
- abnormal circulating cytokine level / MGI
- increased activation-induced B cell apoptosis / MGI
- decreased CD4-positive, alpha-beta memory T cell number / MGI
- postnatal lethality, complete penetrance / MGI
Literature references
- Stroma-mediated dysregulation of myelopoiesis in mice lacking I kappa B alpha.;Rupec Rudolf A, Jundt Franziska, Rebholz Bernd, Eckelt Birgit, Weindl Günther, Herzinger Thomas, Flaig Michael J, Moosmann Sabine, Plewig Gerd, Dörken Bernd, Förster Irmgard, Huss Ralf, Pfeffer Klaus, ;2005;Immunity;22;479-91; 15845452