Percentage and phenotype of iNKT cells in MPS disease patients, and control subjects. (A) Gating strategy to characterize iNKT cells by the expression of CD4, CD8 and CD161 among total T cells. (B) Percentage of iNKT cells in total T cells of MPS II disease patients and controls, (C) CD4+CD8− cells in iNKT cells of MPS II disease patients and controls, (D) CD4−CD8+ cells in iNKT cells of MPS II disease patients and controls, (E) CD8−/double negative (DN) cells in iNKT cells of MPS II disease patients and controls and (F) CD161+ cells in iNKT cells of MPS II and VI disease patients and control group. Horizontal bars represent mean values. * p < 0.05; ** p < 0.01.