Mouse Gene Knockouts
Phenotypes of Mouse Rel/NF-kB/IkB/IKK Knockouts
Most of the genes encoding the transcription factors (Rel/NF-kB), inhibitors (IkB), and kinases (IKK) in the Rel/NF-kB signal transduction pathway have been disrupted in mice.These gene knockouts lead to a variety of phenotypes, which are summarized in the table below.There are also links to the primary references for this information, as on PubMed.
| Gene | Phenotype | Reference | ||
| rela | Embryonic lethal;liver apoptosis;sensitivity to TNF | Beg & Baltimore, 1996 | ||
| c-rel | Impaired T and B cell activation; systemic arthritis resistance | Kontgen et al, 1995Grumont et al, 1998Campbell et al, 2000 | ||
| relb | Defects in acquired and innate immunity; T-cell infiltration of organs; skin inflammation | Burkly et al, 1995Weih et al, 1995Freyschmidt et al, 2007 | ||
| nfkb1 | Immune response defects due to abnormal B-cell response; resistance to arthritis; neural degeneration; sleep irregularities | Sha et al, 1995Campbell et al, 2000Lu et al, 2006; Jhaveri et al, 2006 | ||
| nfkb2 | Abnormal spleen and lymph node architecture; defective T-cell response | Caamano et al, 1998Franzoso et al, 1998 | ||
| c-reldelta C | Lymphoid cell hyperplasia | Carrasco et al, 1998 | ||
| nfkb1
delta C |
Splenomegaly; enlarged lymph nodes; infiltrations of lung & liver; susceptibility to pathogens | Ishikawa et al, 1998 | ||
| nfkb2delta C | Multiple post-natal organ defects | Ishikawa et al, 1997 | ||
| nfkb1/relb | Lethal 3-4 weeks post-natal;Increased organ inflammation | Weih et al, 1997 | ||
| nfkb1/nfkb2 | Reduced growth; cranofacial abnormalities; bone defects; B-cell defects | Franzoso et al, 1997Iotsova et al, 1997 | ||
| nfkb1/c-rel | Decreased humoral immunity | Pohl et al, 2002 | ||
| nfkb1SSAA/SSAA | No T-cell dependent antibody responses | Jacque et al, 2014 | ||
| c-rel/rela | Embryonic lethal early liver apoptosis; skin epidermus development | Grossmann et al, 1999; Gugasyan et al., 2004 | ||
| ikba | Early neonatal lethal inflammatory dermatitis and granulocytosis | Beg et al, 1995Klement et al, 1996 | ||
| ikbb | ||||
| ikbe | Reduction in number of CDD44-CD25+ T cells | Memet et al, 1999 | ||
| bcl-3 | Defects in B- and T-cell responses to antigens | Franzoso et al, 1997Schwartz et al, 1997 | ||
| IKKa | Early neonatal lethal;Skin defects | Hu et al, 1999Takeda et al, 1999 | ||
| IKKb | Embryonic lethal;Liver apoptosis;Sensitivity to TNF; B-cell survival (conditional) | Li et al, 1999Takeda et al, 1999Li et al., 2003 | ||
| IKKa/IKKb | Embryonic lethal; Liver apoptosis; Sensitivity to TNF; Neural defects | Li et al, 2000 | ||
| IKKg (NEMO) | Embryonic lethal; Liver apoptosis; Sensitivity to TNF; Heterozygotes are model for incontinentia pigmenti | Rudolph et al, 2000 Makris et al, 2000 | ||
