Figure 1.
GPI, GPE, GPC, and GPS are elevated in CLN3Δex7-8 mini pigs. Relative levels of 350 lipids and 451 small molecule metabolites were quantified in serum samples from male and female homozygous CLN3Δex7-8 and wild type pigs at 6 (n = 6/group), 24 to 27 (n = 9/group), 36 (n = 10/group), and 48 (n = 3/group) months of age: (A) at 36 months of age, 38 species were significantly upregulated in CLN3Δex7-8 samples including 4 glycerophosphodiesters (red points on graph) and 4 docosahexaenoyl-conjugated phospholipids (red points on graph), (B) chemical structures of glycerophosphodiesters significantly upregulated in CLN3Δex7-8 pigs, (C) chemical structures of phospholipids significantly downregulated in CLN3Δex7-8 pigs, and (D-G) longitudinal plots of glycerophosphodiester levels in wild type and CLN3Δex7-8 pigs. GPI and GPE are significantly elevated at 6, 24 to 27, 36, and 47 to 50 months of age, while GPC and GPS are significantly elevated 36 and 47 to 50 months only. Uncorrected two-tailed homoscedastic t-test in (A). Two-way ANOVA with two-tailed Sidak’s multiple comparisons test in (D) to (G).
*P < .05. **P < .005. ***P < .0005. ****P < .0001.