AUTHOR=Zhuang Haoyang , Szeto Christopher , Han Shuhong , Yang Lijun , Reeves Westley H. 

TITLE=Animal Models of Interferon Signature Positive Lupus

JOURNAL=Frontiers in Immunology

VOLUME=Volume 6 - 2015

YEAR=2015

URL=https://www.frontiersin.org/journals/immunology/articles/10.3389/fimmu.2015.00291

DOI=10.3389/fimmu.2015.00291

ISSN=1664-3224

ABSTRACT=Human lupus is strongly associated with a gene expression signature characterized by over-expression of type I interferon-regulated genes.  A strong interferon signature is not seen in the standard mouse models of lupus, despite considerable evidence for the involvement of toll-like receptor (TLR)-driven interferon production.  In contrast, pristane-induced lupus exhibits a prominent TLR7-dependent interferon signature.  Importantly, genetic disorders with dysregulated interferon production in both humans and mice cause severe autoinflammatory diseases but not the typical manifestations of lupus suggesting that interferon over-production by itself is insufficient to cause SLE.  Murine single-gene models suggest that lupus-like disease may result from abnormalities in B cell activation and the clearance of dead cells.  Pristane may mimic human SLE by causing interferon production along with synergistic abnormalities affecting clearance of apoptotic cells and activation of B cell signaling.