Fig. 4

Lectin staining for sialic acid epitopes in JBTS10 and control skin fibroblasts. a Lectin staining of α-2,6-sialic acid (SNA, green) and α-2,3-sialic acid (MALII, blue) linkages in affected, rescued and control cells, as indicated. Arrows indicate atypical nuclear staining by SNA in UDP-3331. Scale bars 20 µm. b Quantification of the fluorescence signal for SNA in nucleus relative to the entire cell for affected, rescued, and control cells under paraformaldehyde fixation conditions. Error bars represent standard error. Single asterisks indicate p < 0.05; double asterisks indicate p < 0.001 relative to both adult and pediatric male controls (unpaired, heteroscedastic t test). c Comparison of methanol and paraformaldehyde fixation on the relative SNA staining intensity of the nucleus vs. the whole cell. Double asterisks indicate p < 0.001(unpaired, heteroscedastic t test). Error bars represent standard error