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. Author manuscript; available in PMC: 2020 Dec 1.
Published in final edited form as: Nat Immunol. 2019 Oct 21;20(12):1644–1655. doi: 10.1038/s41590-019-0504-0

Fig. 4 |. Human ASM deficiency is associated with reduced CD1d-restricted antigen presentation to iNKT cells and a reduced level of iNKT cells.

Fig. 4 |

a, EBV-transformed B cells were transduced with a lentiviral construct encoding human CD1d, loaded with α-GalCer and cultured with the 58ab hybridoma. The graph shows the mean IL-2 levels from three independent wells with cells from the indicated individuals. The results are representative of three independent experiments. b, Representative dot plots from a patient with NPD and a healthy control. The percentages indicate the percentage of iNKT cells among lymphocytes. c,d, The graphs demonstrate the level of iNKT cells (c) and T cells (d) in patients with NPD (n = 5) compared to healthy controls (n = 70). The values indicate the percentage of iNKT cells or T cells among lymphocytes. e, ASM activity in PBMCs was measured using a colorimetric assay and the iNKT cell phenotype was examined by flow cytometry in healthy controls (n = 25). The graphs show the correlation between ASM activity and the percentage of CD8+ (left) or CD161+ (right) cells among iNKT cells. Mean values are shown with error bars representing the s.e.m. in a, and the line indicates the median value in c. P values were calculated by one-way ANOVA with Bonferroni’s correction for multiple comparisons (a), a two-sided Mann-Whitney U-test (c and d) and two-sided Pearson’s correlation (e). *P < 0.05, **P < 0.01, ***P < 0.001; NS, not significant.