Journal article

Loss of Roquin induces early death and immune deregulation but not autoimmunity

Arianna Bertossi, Martin Aichinger, Paola Sansonetti, Maciej Lech, Frauke Neff, Martin Pal, F Thomas Wunderlich, Hans-Joachim Anders, Ludger Klein, Marc Schmidt-Supprian

Journal of Experimental Medicine | ROCKEFELLER UNIV PRESS | Published : 2011

Abstract

The substitution of one amino acid in the Roquin protein by the sanroque mutation induces a dramatic autoimmune syndrome in mice. This is believed to occur through ectopic expression of inducible T cell co-stimulator (ICOS) and unrestrained differentiation of follicular T helper cells, which induce spontaneous germinal center reactions to self-antigens. In this study, we demonstrate that tissue-specific ablation of Roquin in T or B cells, in the entire hematopoietic system, or in epithelial cells of transplanted thymi did not cause autoimmunity. Loss of Roquin induced elevated expression of ICOS through T cell-intrinsic and -extrinsic mechanisms, which itself was not sufficient to break self..

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University of Melbourne Researchers