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Table 1 Spontaneous models of Sjögren's syndrome

From: Sjögren's syndrome: studying the disease in mice

Strain/modificationa SS-like disease manifestationsb Remarksb Reference
(NZB/NZW)F1 SG and LG infl. SLE-like disease [19]
MRL SG and LG infl. SLE-like disease [29]
    lpr    ↑ SG and LG infl.    lpr exacerbates the MRL disease phenotype [30]
NFS/sld None Aberrant proteolysis of α-fodrin [33]
   3d-Tx    SG and LG infl.    Inflammatory lesions in multiple organs [34]
IQI/Jic    SG and LG infl. Inflammatory lesions in multiple organs [37]
NOD SG and LG infl., ↓ SG and LG function T1D, multiple immune system-related alterations [12]
    H2b    Similar to original NOD strain No T1D [42]
C57BL/6 May develop SG infl. at an old age    Widely used recipient strain  
   NOD-Aec1Aec2    SG and LG infl., ↓ SG and LG function    Increased applicability compared with NOD mice [41]
    Il2-/-    SG and LG infl., ↓ SG function    Generalized lymphoproliferative disease [91]
    Il2Rα-/-    SG and LG infl., ↓ SG function    Generalized lymphoproliferative disease [91]
    Foxp3-/-    None    More susceptible to SS-like disease [94]
   Il10 Tg    SG and LG infl., ↓ SG and LG function    ↑ apoptosis in the SG [95]
   Il14α Tg    SG infl., ↓ SG function    High incidence of CD5+ lymphoma, nephritis [101]
   Baff Tg    SG and LG infl., ↓ SG function    MZ B-cell dominated infl., SLE-like disease [106]
    Tgfβ1-/-    SG and LG infl., ↓ SG function    Poor viability, mixed inflammatory cell response [112]
   Tgfβ1 Tg    ↓ SG function    Impaired SG architecture, SG tissue fibrosis [113]
    Thbs1-/-    LG infl., anti-Ro and anti-La    Detailed assessment of the eye component [116]
    Ar -/-    SG infl.    Estrogen-dependent disease [119]
   RbAp48 Tg    SG and LG infl., ↓ SG function, anti-Ro and anti-La    Assessment of the inflammatory milieu in the SG [122]
    Id3-/-    SG and LG infl., ↓ SG function, anti-Ro and anti-La    Exocrine gland dysfunction precedes SG and LG infl. [125]
    Id3-/-;LckCre    SG and LG infl., ↓ SG function, anti-Ro and anti-La    T-cell lineage specific deletion of Id3 [126]
   r1ΔT/r2n    SG and LG infl., anti-Ro    ↓ Tregs, inflammatory lesions in multiple organs [130]
    IκBαM/M    SG and LG infl., anti-Ro and anti-La    Defective feedback regulation of NF-κB [133]
C57BL/10 May develop SG infl. at an old age C57BL/6 and C57BL/10 diverged prior to 1937  
   NOD-Nss1/Idd5    SG infl.    SG function has not been assessed [82]
SJL SG and LG infl., anti-Ro and anti-La Susceptible to multiple autoimmune diseases [98]
   Il12 Tg    ↑ SG and LG infl., ↓ SG function, ↑ anti-Ro and anti-La    IL-12 exacerbates the SJL disease phenotype [98]
BALB/c None Often used as control strain  
    Act1-/-    SG and LG infl., anti-Ro and anti-La    MZ B-cell dominated infl., SLE-like disease [115]
  1. Selection of murine strains in which Sjögren's syndrome (SS)-like disease manifestations develop spontaneously. ↑, increased; ↓, decreased; Ar, aromatase; 3d-Tz, 3 days after birth thymectomy; infl., inflammation; LG, lacrimal gland; MZ, marginal zone; NOD, nonobese diabetic mice; SG, salivary gland; SLE, systemic lupus erythematosus; T1D, type 1 diabetes; Tg, transgenic; Treg, regulatory T cell. aSpecific modification of the original strain. bFor modified strains, listings refer to relative changes compared with the original, not indented, strain listed above.
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Arthritis Research & Therapy

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