Table 1.

Spontaneous Mouse Models of Lupus

Strain	Parental strains	Lupus-like phenotype specific to the model	References
(NZB x NZW) F1	NZB and NZW	Lymphadenopathy, splenomegaly, high level anti-dsDNA IgG, lupus nephritis (GN). Strong female bias	(Helyer and Howie, 1963)
NZM2410 NZM2328	NZM2410 and NZM2328 are 2 of 27 recombinant inbred strains between NZB and NZW	Similar to (NZB x NZW) F1 with less pronounced female bias	(Rudofsky et al., 1993)
B6.NZM2410.Sle1.Sle2.Sle3	NZM2410 derived genetic loci, Sle1 - 3, are introduced to a B6 non-autoimmune background	Milder phenotypes than the parental NZM2410	(Morel et al., 2000)
MRL/lpr	MRL strain is generated from inbreeding between several strains of mice. The lpr, lymphoproliferation, mutation is a loss of function in the pro-apoptotic Fas gene.	High level of autoAbs: anti-DNA, anti-Sm, rheumatoid factors, GN. Lymphadenopathy contributed mainly by accumulation of CD4− CD8− T cells.	(Cohen and Eisenberg, 1991)
MRL/gld	The gld, generalized lymphoproliferative disease, mutation is a loss of function mutation in the FasL gene.	Lymphadenopathy, autoAbs, GN.
BXD2	C57BL/6J x DBA/2J recombinant inbred strain	High level of IL - 17, autoAbs (anti-DNA, anti-histone, and rheumatoid factor), GN and arthritis.	(Hsu et al., 2008)
BXSB/Yaa	(B6 x SB/Le) F1 x SB/Le --> Inbreeding. Yaa, Y-linked autoimmune accelerator, refers to the translocation of 16 genes from the X chromosome, including TLR7 onto the Y chromosome	Only males are affected. AutoAbs skewed toward RNA-specificities, monocytosis.	(Murphy and Roths, 1979; Santiago-Raber et al., 2008)