Download nfkb_gene_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
rela
c-rel
relb
nfkb1
nfkb2
c-rel
delta C
nfkb1
delta C
nfkb2
delta C
nfkb1/relb
nfkb1/nfkb2
nfkb1/c-rel
c-rel/rela
ikba
Phenotype
Reference
Embryonic lethal;
Beg & Baltimore, 1996
liver apoptosis;
sensitivity to TNF
Impaired T and B cell
Kontgen et al, 1995
activation; systemic arthritis Grumont et al, 1998
resistance
Campbell et al, 2000
Defects in acquired and innate Burkly et al, 1995
immunity; T-cell infiltration Weih et al, 1995
of organs; skin inflammation
Freyschmidt et al, 2007
Immune response defects due Sha et al, 1995
to abnormal B-cell response; Campbell et al, 2000
resistance to arthritis; neural
degeneration; sleep
Lu et al, 2006; Jhaveri et al,
irregularities
2006
Abnormal spleen and lymph Caamano et al, 1998
node architecture; defective Franzoso et al, 1998
T-cell response
Lymphoid cell hyperplasia
Carrasco et al, 1998
Splenomegaly; enlarged
Ishikawa et al, 1998
lymph nodes; infiltrations of
lung & liver; susceptibility to
pathogens
Multiple post-natal organ
Ishikawa et al, 1997
defects
Lethal 3-4 weeks post-natal; Weih et al, 1997
Increased organ inflammation
Reduced growth; cranofacial
abnormalities; bone defects;
B-cell defects
Decreased humoral immunity
Embryonic lethal early liver
apoptosis; skin epidermus
development
Early neonatal lethal
inflammatory dermatitis and
granulocytosis
Franzoso et al, 1997
Iotsova et al, 1997
Pohl et al, 2002
Grossmann et al, 1999;
Gugasyan et al., 2004
Beg et al, 1995
Klement et al, 1996
ikbb
ikbe
bcl-3
IKKa
IKKb
IKKa/IKKb
IKKg (NEMO)
Reduction in number of
CDD44-CD25+ T cells
Defects in B- and T-cell
responses to antigens
Early neonatal lethal;
Skin defects
Embryonic lethal;
Liver apoptosis;
Sensitivity to TNF; B-cell
survival (conditional)
Embryonic lethal; Liver
apoptosis; Sensitivity to TNF;
Neural defects
Embryonic lethal; Liver
apoptosis; Sensitivity to TNF;
Heterozygotes are model for
incontinentia pigmenti
Memet et al, 1999
Franzoso et al, 1997
Schwartz et al, 1997
Hu et al, 1999
Takeda et al, 1999
Li et al, 1999
Takeda et al, 1999
Li et al., 2003
Li et al, 2000
Rudolph et al, 2000 Makris et
al, 2000