Fig. 5: Mitochondrial stress develops in Rpgr mutant photoreceptors.

a, b VDAC1 expression is increased in mutant photoreceptor inner segments at 12 (a) and 18 (b) months compared to wild-type littermate controls (scale bar = 25 μm. c Quantification of VDAC1 puncta at 12 and 18 months (N = 5 animals per genotype, except wild type at 18 months where n = 3 animals); error bars show SEM; *p = 0.01 (12 months), p = 0.039 (18 months) by unpaired t-test). d Mitochondrial stress assay shows basal oxygen consumption rate (OCR) is reduced in RpgrEx3d8 mutants at 14 months. Mean OCR for 4 biological replicates (n = 4 animals per genotype; 10 biopsies per animal) is shown at each time point (error bars show SD). Vertical lines indicate points of addition of relevant modulators (oligo oligomycin, FCCP carbonyl cyanide p-trifluoro-methoxyphenyl hydrazone; R/AA rotenone + antimycin A). e Mean OCR of mutant retina (time point 3 = Basal, time point 7 = Max) normalised to the mean OCR of wild-type littermate controls at the same time point (error bars show SD; **p = 0.009 by t-test). Source data are provided as a Source Data file.