Initial characterization of Roquin-defective mice (Roquinsan/san), which have an M199R mutation in the Roquin protein, revealed chronic inflammation consisting of lymphadenopathy, splenomegaly, thrombocytopenia, necrotizing hepatitis, and increased numbers of CD4+ follicular T cells and ICOS expression [3], [4]. The gene discussed is RC3H1; the disease is Lymphadenopathy.