SATB1 Conditional Knockout Results in Sjögren's Syndrome in Mice

J Immunol. 2017 Dec 15;199(12):4016-4022. doi: 10.4049/jimmunol.1700550. Epub 2017 Nov 10.


Sjögren's syndrome (SS) is an autoimmune disease in which exocrine tissues are affected by cellular and humoral immunity. As a result, the salivary and lacrimal glands of patients with SS are damaged, leading to xerostomia (dry mouth) and keratoconjunctivitis sicca (dry eyes). Because experimental approaches to investigate SS pathogenesis in human patients are limited, development of a mouse model is indispensable for understanding the disease. In this study, we show that special AT-rich sequence binding protein-1 conditional knockout (SATB1cKO) mice, in which the SATB1 gene is specifically deleted from hematopoietic cells, develop SS by 4 wk of age, soon after weaning. Female mice presented an earlier onset of the disease than males, suggesting that female SATB1cKO mice are more susceptible to SS. T cell-dominant immune cell infiltration was observed in the salivary glands of 4 wk old SATB1cKO mice, and the frequency of B cells gradually increased as the mice aged. Consistently, levels of anti-SSA and anti-SSB Abs were increased around 8 wk of age, after salivary production reached its lowest level in SATB1cKO mice. These results suggest that SATB1cKO mice can be a novel SS model, in which the progression and characteristics of the disease resemble those of human SS.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, N.I.H., Extramural

MeSH terms

  • Adoptive Transfer
  • Animals
  • B-Lymphocytes / immunology
  • Disease Models, Animal*
  • Disease Progression
  • Female
  • Genetic Predisposition to Disease
  • Lupus Nephritis / etiology
  • Lupus Nephritis / immunology
  • Lupus Nephritis / pathology
  • Male
  • Matrix Attachment Region Binding Proteins / deficiency*
  • Matrix Attachment Region Binding Proteins / physiology
  • Mice
  • Mice, Inbred C57BL
  • Mice, Knockout
  • Salivary Glands / immunology
  • Salivary Glands / pathology
  • Salivation
  • Sjogren's Syndrome / genetics*
  • Sjogren's Syndrome / immunology
  • T-Lymphocytes / immunology
  • T-Lymphocytes, Regulatory / immunology


  • Matrix Attachment Region Binding Proteins
  • Satb1 protein, mouse