Table 2.
Phenotypic characteristics of ERdj5−/− mice and other mouse SS models.
| Inflammatory infiltrates in SG | SG tissue damage | Serum AutoAbs | Salivary hypofunction | Female predilection | Cytokines in serum | Cytokines in SGs | Parenchymal inflammation | Other phenotypes | ||
|---|---|---|---|---|---|---|---|---|---|---|
| Knock-out | ERdj5 KO | + B-cells, T-cells (2:1 ratio) |
+ | + in females anti-Ro anti-La |
+ | + | Elevated ♀: IL-23, IL-18 ♂: IL-17A |
Elevated ♀: IL-23, IL-10 |
Kidney, occasional infiltrations in liver | nd |
| Id3 KO | + IFN-γ and IL-4 producing lymphocytes |
nd | + Anti-Ro, Anti-La, Autoreactivity to ductal cells |
+ | – | nd | nd | Occasional infiltrations in kidney and lung | Lachrymal gland inflammation and hypofunction. Tumors in numerous organs. Late onset of SS-like phenotype. Skin lesions by over-scratching | |
| IkB-ζ KO | – | – | + Anti-Ro, Anti-La |
nd | nd | nd | nd | Interstitial pneumonia | Lachrymal gland inflammation, apoptosis, and hypofunction. Periocular dermatitis. Conjunctivitis. Splenomegaly. Lymphadenopathy. Elevated serum IgGs | |
| Transgenic-overexpression | BAFF | + B-cells predominantly |
+ | - for Anti–Ro- for Anti-La | + | – | nd | nd | Late onset nephritis. Occasional lung, liver infiltrations | SLE-like. SS-like with age. Reduction in T-regs. Germinal centers in SGs. Hyperactive B-cells |
| IL-12 in SJL strain |
+ | nd | + ANAs, Anti-La |
+ | + | nd | nd | Lung inflammation | Lachrymal gland inflammation. Mild autoimmune thyroiditis. Affected osmoregulation of SGs | |
| Chimeric | SS PBMCs in NOD-scid IL-2rγ (null) | + CD4 predominantly |
nd | nd | + | nd | Elevated IFN-γ, IL-10, IL-17, IL-2, IL-6, TNFα | nd | nd | Type-I diabetes. Lachrymal gland inflammation |
| Spontaneous models | MRL/lpr | + IFN-γ and IL-17 producing CD4+ |
nd | + Anti-Ro, Anti-La |
– | – for SG+ for lachrymal |
nd | IFN γ, IL-1β, IL-6, TNFα | nd | SLE. Lachrymal gland inflammation. Early death. Aggressive autoimmune lympho-proliferative disorder |
| NOD | + Dynamic CD4+ PD1+ FOXP3-, mainly Th1 |
+ | + Anti-Ro, Anti-La |
+ | + SG in ♀ Lachr. in ♂ |
IFNs | IL-1β, IL-2, TNFα, IFN-γ, IL-10, IL-17, IL-12(p40) | Lung inflammatory infiltrations. Kidney antibody deposition | IDDM. Thyroiditis-like. SLE. Myasthenia. SS phenotype affected by housing conditions. Weak association with HLA haplotypes | |
| NOD Aec1. Aec2 | + Progressive T-cell and B-cell infiltrations |
+ | + | + | + | Differentially expressed ± IFNs | ± IFNs. Elevated cytokines | nd | Anti-M3R antibodies. Lachrymal gland hypofunction in females | |
| NZB/NZW F1 | Augmented by incomplete Freund's adjuvant IP injection (F.adj. inj.) | nd | ANAs and anti-Ro augmented by F.adj.IP | Augmented by F.adj. and polyI:C injection | + | nd | Augmented by polyI:C injection | Kidney | SLE-like phenotype. Non-specific inflammatory stimuli. Conjunctivitis | |
| Immunization | Ro60 in BALB/c | + | nd | nd | + | nd | nd | nd | nd | Anti-Ro, Anti La in saliva. Late onset. Requires multiple immunizations |
| Alum in NZM2758 | + | nd | + ANAs | + | nd | nd | “Inflammasome components” | nd | “P2X7 receptor-dependent phenotypes” |
+, SS trait is reported in the relevant literature; –, SS trait reported negative in the relevant literature; nd, not determined in the relevant literature; italics, trait discussed as possibility but not shown in experimental data.