Stabilized β -Catenin Ameliorates ALPS-Like Symptoms of B6/ lpr Mice

Autoimmune lymphoproliferative syndrome (alps) is an incurable disease mainly caused by the defect of fas-mediated apoptosis and characterized by nonmalignant autoimmune lymphoproliferation. stabilized β-catenin could not only potentiate fas-mediated t cell apoptosis via upregulating the expression of fas on activated t cells,but also potentiate t cell apoptosis via intrinsic apoptotic pathway. in the present study,we introduced β-cattg into lpr/lpr mice and aimed to explore the potential role of stabilized β-catenin (β-cattg) in the development of alps-like phenotypes of lpr/lpr mice. we found that the total splenocyte cells and some compositions were slightly downregulated in β-cattglpr/lpr mice,especially the cd4 and cd8 tem cells were significantly reduced. meanwhile,stabilized β-catenin obviously decreased the numbers of spleen tcrβ+cd4-cd8- t (dnt) cells,and the levels of some serum proinflammatory factors also were lowered in β-cattglpr/lpr mice. beyond that,stabilized β-catenin slightly lowered the levels of the serum autoantibodies and the scores of kidney histopathology of β-cattglpr/lpr mice compared with lpr/lpr mice. our study suggested that stabilized β-catenin ameliorated some alps-like symptoms of lpr/lpr mice by potentiating fas-independent signal-mediated t cell apoptosis,which might uncover a potential novel therapeutic direction for alps. © 2017 xiaoxie xu et al.