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. 2024 Oct 22;25(21):11361. doi: 10.3390/ijms252111361

Figure 1.

Figure 1

Biosynthetic forms of NPC1 proteins in different patients. The cellular lysates from fibroblasts derived from a healthy donor (WT), patient 1 (P1), patient 2 (P2), patient 3 (P3), and patient 4 (P4) were immunoprecipitated with anti-NPC1 and further treated or not treated with endo H. The mature complex glycosylated endo H-resistant form appears as a 190 kDa band and the endo H-sensitive immature form is cleaved to a 130 kDa band. The immunoprecipitates were subjected to SDS-PAGE and Western blot for NPC1 analysis (A). The band intensities of the complex and mannose-rich forms of the NPC1 protein were quantified and presented as percentage of the total (B). Equal protein amounts of cellular lysates were subjected to SDS-PAGE for NPC1 expression analysis versus the house keeping protein, actin (C). Ordinary one-way ANOVA, ns p > 0.05, * p < 0.05, and **** p < 0.0005 versus WT, S.E.M., and n = 3.