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. Author manuscript; available in PMC: 2013 Oct 28.
Published in final edited form as: Arch Immunol Ther Exp (Warsz). 2009 Feb 14;57(1):10.1007/s00005-009-0002-4. doi: 10.1007/s00005-009-0002-4

Table 2. Mouse models of Sjögren's syndrome-like disease.

Mouse strains Characteristics Disease manifestation/phenotypes
MRL/lpr Mutation of lpr gene that encodes Fas protein

  • Diffuse lymphocytic infiltration of glands, presence of auto-antibodies (against ssDNA, RNPs, IgG), but no loss of secretion or detection of anti-M3R antibodies

  • Develop dacryoadenitis of the lacrimal glands and sialadenitis of the submandibular, parotid, and lingual glands
NOD Spontaneous insulitis and diabetes SjS-like disease phenotype with loss of secretion, anti-M3R antibodies, and focal lymphocytic infiltration
NOD.B10-H2b NOD with H2b from C57BL/10 SjS-like disease phenotypes without type I diabetes
C57BL/6.NOD-Aec1Aec2 C57BL/6 mouse carrying Aec1 (Idd3) genetic region on Chr. 3 and Aec2 (Idd5) genetic region on Chr. 1 from NOD mouse

  • SjS-like disease phenotypes

  • Increased expression of caspase, matrix metalloproteinase and PSP proteolytic enzymes, increased apoptosis, and altered protein secretions

  • Activated T/B cells, increased expression of pro-inflammatory cytokines, appearance of autoantibodies and loss of secretory function
(NZB/NZW) F1 Naturally occurring mouse model by crossing NZB and NZW Lacrimal gland involvement that shows greater percentage of B cells compared with MRL/lpr mouse
NFS/sld Autosomal recessive gene with sublingual gland differentiation arrest Anti-α-fodrin antibodies
Id3 gene knock-out (KO) No Id3 (basic helix-loop-helix transcription factor) production, a dominant negative inhibitor of gene expression Impaired TCR-mediated T-cell selection, loss of secretion and presence of anti-Ro and anti-La antibodies
Aromatase gene KO Estrogen deficient due to absence of enzyme catalyzing the conversion of testosterone to estradiol B cell hyperplasia in the BM and spleen and anti-α-fodrin antibodies
Baff gene knock-in Over-expression of the B-cell survival factor, BAFF (BLys) Lymphocytic infiltration with a majority of B cells, leading to loss of secretion by 15–17 months of age
IQI/Jic Inbred strain originating from ICR with SjS-like disease in the absence of diabetes Anti-kallikrein-1 and -13 antibodies