OBJECTIVE: Autoimmune lymphoproliferative syndrome (ALPS) is a disorder of apoptosis,
often presenting in childhood. Similarly, MRL/lpr−/− mice homozygous for Fas mutations
develop an ALPS-like disease with autoimmunity, lymphadenopathy, splenomegaly, and
expansion of double-negative T cells. Currently, there are no proven therapies with
adequate safety margins for sustained abolition of the lymphoproliferation associated with
ALPS. We sought to test the ability of valproic acid (VPA), a histone deacetylase inhibitor, to …