Table 1.

Overview of CBM-deficient mouse models.

Feature	Card11−/−	Card11un/un	Bcl10−/−	Malt1−/−	Malt1PD/PD
B CELLS
Populations
Total	Normal	Normal	Normal	Normal	Normal
FO	Normal	Normal	↓	Normal	Normal
MZ	↓	Normal	↓	↓	↓
B1	↓	↓	↓	↓	↓
GC	ND	ND	ND	↓	↓
Proliferation
IgM	↓	↓	↓	↓	Normal
CD40	↓	↓	↓	↓	ND
CD40L	ND	ND	ND	↓	↓
IgM+CD40	↓	ND	↓	ND	ND
LPS	↓	Normal	Normal	↓ or normal	↓ or normal
IgM+IL-4	ND	↓	ND	↓	↓
CD40+IL-4	ND	↓	ND	↓ or normal	Normal
Signaling
IκBα/NF-κB	↓	↓	↓	↓ or normal	Normal
JNK	↓	↓	↓	↓ or normal	↓ or normal
ERK	Normal	Normal	Normal	Normal	Normal
Tyrosine	Normal	Normal	ND	Normal	ND
Calcium	Normal	Normal	Normal	ND	ND
T CELLS
Populations
Total	Normal	Normal	Normal	Normal	Normal
CD4	Normal	Normal	Normal	Normal	Normal
CD8	Normal	Normal	Normal	Normal	Normal
Treg	↓	↓	↓	↓	↓
Th1	↓	Normal	ND	↑ or normal	↑
Th2	↓	↑	ND	↑ or normal	↑
Th17	↓	Normal	ND	↓ or normal	↑
Tfh	ND	ND	ND	↓	↓
NKT	Normal	ND	Normal	ND	ND
DN3	↓	ND	ND	↓	ND
DN4	↑	ND	↑	↑	↑
DP	ND	ND	↓	Normal	Normal
Proliferation
CD3	↓	Normal	↓	↓	↓
CD3+CD28	↓	↓	↓	↓	↓
P/I	↓	ND	↓	↓	ND
Signaling
IκBα/NF-κB	↓	ND	↓	↓	Normal
JNK	↓	ND	ND	↓	↓ or normal
ERK	Normal	ND	Normal	Normal	Normal
Tyrosine	Normal	ND	Normal	Normal	ND
Calcium	Normal	ND	Normal	ND	ND
Activation Markers (α-CD3+CD28)
CD25	↓	↓	↓	↓	Normal
CD69	↓	↓	↓	↓	ND
CD40L	ND	↓	ND	ND	ND
OX40	ND	↓	ND	ND	ND
ICOS	ND	↓	ND	ND	ND
CD44	↓	ND	↓	↓	ND
IMMUNOGLOBULINS
Basal
IgM	↓	↓	↓	↓	↓
IgG1	↓	Normal	↓	↓	↑
IgG2a	↓	ND	↓	↓	↓
IgG2b	↓	Normal	↓	↓	↓
IgG3	↓	↓	↓	↓	↓
IgA	↓	ND	↓	↓ or normal	Normal
IgE	ND	↑	ND	ND	↑
T-dependent
IgM	↓	↓	↓	↓	↓
IgG1	↓	↓	↓	↓	↓
IgG2a	↓	ND	↓	ND	ND
IgG2b	ND	ND	↓	ND	ND
IgG3	ND	ND	↓	ND	ND
Other immune manifestations	None	Atopy and dermatitis with age	None	None	Spontaneous multi-organ inflammation
References	(11, 12, 81–84)	(13, 17, 85)	(14, 15, 86, 87)	(16, 88–90)	(89, 91–93)

Lymphocyte characteristics, immunoglobulin levels, and additional immune features are summarized and compared for Card11^−/−, Card11^un/un, Bcl10^−/−, Malt1^−/−, Malt1^PD/PD mice. ND, no data; ↑, increased levels relative to normal range; ↓, decreased levels relative to normal range; FO, follicular B cell; MZ, marginal zone B cell; GC, germinal center B cell; LPS, lipopolysaccharide; IL-4, interleukin 4; IκBα, NF-κB inhibitor alpha; NF-κB, nuclear factor kappa B; Treg, T regulatory cell; Th, T helper cell; Tfh, T follicular helper cell; NKT, natural killer T cell; DN, double negative; DP, double positive; P/I, phorbol 12-myristate 13-acetate (PMA) / ionomycin; JNK, c-Jun N-terminal kinase; ERK, extracellular signal-regulated kinase; ICOS, inducible T cell costimulatory.