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. 2023 Mar 15;133(6):e161944. doi: 10.1172/JCI161944

Figure 4. 1-TbAd induces storage of known substrates in lysosomal storage diseases.

Figure 4

(A) Unknown lipids could be linked on a 1-to-1 basis with TAGs (dashed arrow) based on retention time (~19 s) and mass (13.979 mu) increments, which correspond to an ether linkage substituting an ester linkage, suggesting that the unknowns were MADAGs. (B) The MADAG structure was confirmed by CID-MS. (C) After quantitation using TAG as the external standard, the dose response to 1-TbAd of 4 MADAGs with the indicated chain length and saturation pattern was reported. P values were determined by 1-way ANOVA followed by post test for linear trend. (D) The 1-TbAd–induced hexosylceramide in macrophages matches the structure of β-glucosylceramide, based on CID-MS and coelution with an authentic internal standard. (E) A C42:2 dihexosylceramide induced by 1-TbAd was solved as LacCer, based on CID-MS and coelution with an authentic standard. The P values for A, C, and E were determined by 1-way ANOVA followed by Dunnett’s multiple-comparison test.