Study on mechanisms of autoantibody production in the pathophysiology of lysosomal storage disorders

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In this study we focused on thymic event from the point of morphology, thymic subpopulation, and gene expression to see the autoimmune mechanisms happening in Sandhoff disease (SD) mice. Thymus from SD mice greater than 15weeks of age showed marked decrease in the percentage of immature T cells and significantly increased CD4+ T cells. During the involution, apoptotic thymic cells and IgG deposition to T cells were increased. CXCL13, one of these genes, was expressed specifically in the thymus, and B1 cells were increased in the thymus. These results suggest that in SD mice it may convert the usually poorly immunogenic thymus into an organ prone to induce autoimmunity, including chemotaxis of B1 cells toward CXCL13.