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. 2016 Dec 24;56(Suppl 1):i88–i99. doi: 10.1093/rheumatology/kew400

Table 1.

Main mouse models of SLE and their characteristics

Lupus models Main genetic components Related human genetic associations Immunological characteristics Model used to assess
Spontaneous

  • NZB/W

  • related: NZM2410

  • NZM2328

  • B6.Sle123

  • Locuses:

  • NZM2410 derived: Sle1–3

  • NZM2328 derived:

  • Cgnz1, Agnz1, Adnz1

 Ccr2, FcgRIIA, FcgRIIIA, FcgRIIIb, PARP, CRP/SAP, kallikrein genes, possibly SLAMF genes

  • Splenomegaly

  • GN (subacute to chronic)

  • Moderate ANAs, high anti-dsDNA antibodies

  • Persistence of long-lived plasma cells

  • Weak IFN signature

  • Immune dysregulation

  • Chronic kidney disease (acute and chronic in NZM2328)

  • Endothelial and cardiac effects
MRL/lpr Fas mutation (lpr) Fas/FasL polymorphisms

  • Lymphoproliferation

  • Splenomegaly

  • Extremely enlarged lymph nodes

  • GN (subacute proliferative)

  • High ANAs, high anti-dsDNA antibodies, high anti-snRNP antibodies

  • Expansion of CD4−CD8−CD3+ T cells

  • No IFN signature

  • Immune dysregulation

  • Kidney disease

  • Cutaneous lupus

  • Neurological manifestations

  • Arthritis

  • BXSB

  • related:

  • B6.TLR7.Tg

  • B6.Sle1Tg7

  • Locuses:

  • Bxsb1–6

  • Yaa

  • TLR7 upregulation

  • TLR7 copy number variations,

  • TLR7 polymorphisms,

  •  polymorphisms of TLR7- signalling pathways (i.e. IRF5)

  • Splenomegaly

  • GN (acute proliferative)

  • Monocytosis

  • Moderate ANAs, moderate anti-dsDNA antibodies (high anti-snRNP antibodies in B6.Sle1Tg7)

  • Weak IFN signature in the kidney

  • Immune dysregulation

  • Kidney disease (acute)
Accelerated

  • IFNα accelerated (various strains, i.e. NZB/W, B6.Sle123, NZM2328)

 Strain dependent

  • Strain dependent,

  • IFN signature

  • Accelerated disease, with characteristics of the conventional strain

  • Highly reproducible, rapid-onset GN without increased leucocyte infiltration

  • T cell dependent

  • Strong IFN signature

  • Immune dysregulation

  • Acute severe kidney disease

  • Drug resistance attributable to IFN signature
Induced Pristane induced (BALB/c, C57BL/6, DBA/1, SJL) Pristane induces lupus in mice lacking genetic predisposition

  • IFN signature,

  • TLR7 dependent

  • Rapid-onset, severe disease

  • High anti-RNP, anti-Sm, anti-dsDNA antibodies

  • Strong IFN signature

  • Immune dysregulation

  • Acute severe kidney disease