IMPC phenotypes (gene matching)
- preweaning lethality, incomplete penetrance / IMPC
B6.Cg-Ikbkbtm2Cgn/Cnrm
| Status | Available to order |
| EMMA ID | EM:01921 |
| International strain name | B6.Cg-Ikbkbtm2Cgn/Cnrm |
| Alternative name | IKK2FL (IKK2floxed) |
| Strain type | Targeted Mutant Strains : Conditional mutation |
| Allele/Transgene symbol | Ikbkbtm2Cgn |
| Gene/Transgene symbol | Ikbkb |
Information from provider
| Provider | Klaus Rajewsky |
| Provider affiliation | CBR Institute for Biomedical Research, Immune Disease Institute |
| Genetic information | The Ikbkb (IKK2) targeting vector was constructed by placing a 2kb EcoRI-BglII genomic fragment, containing exons 6 and 7, between the loxP-flanked PGKneo cassette and the third loxP site. An upstream 2.6 kb ScaI-EcoRI fragment and a 4.2 kb downstream BglII fragment were used as arms of homology. Bruce 4 ES cells, derived from B6.Cg-Thy1a mice, were cultured, transfected and selected as previously described. Homologous recombinant clones were isolated and the loxP flanked PGKneo cassette was excised by transient expression of cre recombinase. Germ-line transmitting chimeras were generated using ES cells carrying the floxed Ikbkb gene. |
| Phenotypic information | Upon cre recombinase-mediated deletion of exons 6 and 7, which correspond to nucleotides 389-567 of the coding Ikbkb (IKK2) cDNA sequence, premature termination codons are introduced, thus producing an Ikbkb null allele. |
| References |
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Information from EMMA
| Archiving centre | CNR, Consiglio Nazionale delle Ricerche, Monterotondo, Italy |
Disease and phenotype information
Orphanet associated rare diseases, based on orthologous gene matching
- Severe combined immunodeficiency due to IKK2 deficiency / Orphanet_397787
MGI phenotypes (gene matching)
- small liver / MGI
- abnormal hepatocyte morphology / MGI
- dissociated hepatocytes / MGI
- no abnormal phenotype detected / MGI
- increased susceptibility to viral infection / MGI
- abnormal B cell physiology / MGI
- liver degeneration / MGI
- increased hepatocyte apoptosis / MGI
- liver hemorrhage / MGI
- abnormal response to infection / MGI
- abnormal cell physiology / MGI
- abnormal CD8-positive, alpha-beta T cell differentiation / MGI
- decreased interleukin-6 secretion / MGI
- prenatal lethality, complete penetrance / MGI
- embryonic lethality during organogenesis, complete penetrance / MGI
- lethality throughout fetal growth and development, complete penetrance / MGI
- increased fibroblast apoptosis / MGI
- increased response to antigen / MGI
Literature references
- FDC-specific functions of p55TNFR and IKK2 in the development of FDC networks and of antibody responses.;Victoratos Panayiotis, Lagnel Jacques, Tzima Sotiria, Alimzhanov Marat B, Rajewsky Klaus, Pasparakis Manolis, Kollias George, ;2006;Immunity;24;65-77; 16413924
- TNF-mediated inflammatory skin disease in mice with epidermis-specific deletion of IKK2.;Pasparakis Manolis, Courtois Gilles, Hafner Martin, Schmidt-Supprian Marc, Nenci Arianna, Toksoy Atiye, Krampert Monika, Goebeler Matthias, Gillitzer Reinhard, Israel Alain, Krieg Thomas, Rajewsky Klaus, Haase Ingo, ;2002;Nature;417;861-6; 12075355
- IkappaB kinase signaling is essential for maintenance of mature B cells.;Pasparakis Manolis, Schmidt-Supprian Marc, Rajewsky Klaus, ;2002;The Journal of experimental medicine;196;743-52; 12235208
- Mature T cells depend on signaling through the IKK complex.;Schmidt-Supprian Marc, Courtois Gilles, Tian Jane, Coyle Anthony J, Israël Alain, Rajewsky Klaus, Pasparakis Manolis, ;2003;Immunity;19;377-89; 14499113
- Inhibition of NF-kappaB activation in macrophages increases atherosclerosis in LDL receptor-deficient mice.;Kanters Edwin, Pasparakis Manolis, Gijbels Marion J J, Vergouwe Monique N, Partouns-Hendriks Iris, Fijneman Remond J A, Clausen Björn E, Förster Irmgard, Kockx Mark M, Rajewsky Klaus, Kraal Georg, Hofker Marten H, de Winther Menno P J, ;2003;The Journal of clinical investigation;112;1176-85; 14561702
- Differential dependence of CD4+CD25+ regulatory and natural killer-like T cells on signals leading to NF-kappaB activation.;Schmidt-Supprian Marc, Tian Jane, Grant Ethan P, Pasparakis Manolis, Maehr René, Ovaa Huib, Ploegh Hidde L, Coyle Anthony J, Rajewsky Klaus, ;2004;Proceedings of the National Academy of Sciences of the United States of America;101;4566-71; 15070758
- I kappa B kinase 2 deficiency in T cells leads to defects in priming, B cell help, germinal center reactions, and homeostatic expansion.;Schmidt-Supprian Marc, Tian Jane, Ji Hongbin, Terhorst Cox, Bhan Atul K, Grant Ethan P, Pasparakis Manolis, Casola Stefano, Coyle Anthony J, Rajewsky Klaus, ;2004;Journal of immunology (Baltimore, Md. : 1950);173;1612-9; 15265889
- Deletion of IKK2 in hepatocytes does not sensitize these cells to TNF-induced apoptosis but protects from ischemia/reperfusion injury.;Luedde Tom, Assmus Ulrike, Wüstefeld Torsten, Meyer zu Vilsendorf Andreas, Roskams Tania, Schmidt-Supprian Mark, Rajewsky Klaus, Brenner David A, Manns Michael P, Pasparakis Manolis, Trautwein Christian, ;2005;The Journal of clinical investigation;115;849-59; 15776110
- IKK mediates ischemia-induced neuronal death.;Herrmann Oliver, Baumann Bernd, de Lorenzi Rossana, Muhammad Sajjad, Zhang Wen, Kleesiek Jens, Malfertheiner Max, Köhrmann Martin, Potrovita Ioana, Maegele Ira, Beyer Cordian, Burke James R, Hasan Mazahir T, Bujard Hermann, Wirth Thomas, Pasparakis Manolis, Schwaninger Markus, ;2005;Nature medicine;11;1322-9; 16286924
- Localized inflammatory skin disease following inducible ablation of I kappa B kinase 2 in murine epidermis.;Stratis Athanasios, Pasparakis Manolis, Markur Doreen, Knaup Renate, Pofahl Ruth, Metzger Daniel, Chambon Pierre, Krieg Thomas, Haase Ingo, ;2006;The Journal of investigative dermatology;126;614-20; 16397523
- Berberine functions as a negative regulator in lipopolysaccharide -induced sepsis by suppressing NF-κB and IL-6 mediated STAT3 activation.;Wang Yin, Du Pengfei, Jiang Donghui, ;2020;Pathogens and disease;78;eaat5401; 32857851
- GLK-IKKβ signaling induces dimerization and translocation of the AhR-RORγt complex in IL-17A induction and autoimmune disease.;Chuang Huai-Chia, Tsai Ching-Yi, Hsueh Chia-Hsin, Tan Tse-Hua, ;2018;Science advances;4;; 30214937