Sjögren's syndrome: studying the disease in mice

Delaleu, Nicolas; Nguyen, Cuong Q; Peck, Ammon B; Jonsson, Roland

doi:10.1186/ar3313

Arthritis Research & Therapy

Table 1 Spontaneous models of Sjögren's syndrome

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

Strain/modification^a	SS-like disease manifestations^b	Remarks^b	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]
H2^b	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	↓ T_regs, inflammatory lesions in multiple organs	[130]
IκBα^M/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]

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; T_reg, 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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ISSN: 1478-6362

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