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. 2019 Aug 14;9:11829. doi: 10.1038/s41598-019-47352-9

Figure 5.

Figure 5

Defects in the mitochondrial presequence import pathway in fibroblasts from PD patients with PARK2 and PINK1 mutations are rescued by phosphomimetic ubiquitin. (A–F) Mean bioluminescent signal for Probe 1 in primary fibroblasts (A–C,E–H) or iPSC-derived neurons (D) from control individuals and PARK2/PINK1 patients (A), a subset of or single PARK2 patients (B–D,G,H), or PINK1 patients (E–H), showing a weaker signal in PINK1 patients and PARK2 Patients 1-3, associated with presence of the partially functional Parkin R42P substitution, and rescue effects of Parkin, PINK1, PINK1 E240K or Ub S65D/A overexpression. The numbers on the graphs indicate the mean signals for individual patients, as presented in Supplementary Table 1, with n = 4–6 independent experiments (A,B,E), n = 4–5 wells from one experiment representative of three (C,F), or n = 4–10 wells from one of 2–5 independent experiments (D,G,H). Note that the overproduction of Parkin, PINK1 or phosphomimetic Ub S65D partially normalizes the signal in fibroblasts from individual PARK2 or PINK1 patients, whereas PINK1 E240K and Ub S65A do not have any effect. Results are expressed as means ± SEM. *p < 0.05, **p < 0.01, ***p < 0.001 versus control individuals (A,B,D,E, Student’s t-test) or the conditions indicated (C,F–H, one-way ANOVA with Dunnett’s (C,F) or Holm-Sidak’s (G,H) posthoc tests).