REAGENTS

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, 1995
Grumont et al, 1998
Campbell et al, 2000
relb
Defects in acquired and innate immunity; T-cell infiltration of organs; skin inflammation Burkly et al, 1995
Weih et al, 1995

Freyschmidt et al, 2007

nfkb1
Immune response defects due to abnormal B-cell response; resistance to arthritis; neural degeneration; sleep irregularities Sha et al, 1995
Campbell et al, 2000

Lu et al, 2006; Jhaveri et al, 2006

nfkb2
Abnormal spleen and lymph node architecture; defective T-cell response Caamano et al, 1998
Franzoso et al, 1998
c-rel
delta 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
nfkb2
delta 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, 1997
Iotsova et al, 1997
nfkb1/c-rel
Decreased humoral immunity Pohl et al, 2002
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, 1995
Klement 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, 1997
Schwartz et al, 1997
IKKa
Early neonatal lethal;
Skin defects
Hu et al, 1999
Takeda et al, 1999
IKKb
Embryonic lethal;
Liver apoptosis;
Sensitivity to TNF; B-cell survival (conditional)
Li et al, 1999
Takeda et al, 1999

Li 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