Fig. 1. Treatment with the chaperone 4-PBA improves P23H-rhodopsin folding and reduces ERS.

a Representative western blots probed for rhodopsin (RHO) in the soluble and insoluble fraction of total retinal protein from P23H mice after 2 weeks of treatment with 4-PBA or vehicle-only as control (CTL). b Quantification of western-blot band intensities of soluble and insoluble RHO normalized to total RHO, and ratio of soluble RHO to insoluble RHO. Measured areas are indicated by the red rectangles in a. Statistical analyses with unpaired t-test. c Representative western blots probed for CHOP, BIP, and loading control GAPDH in retinas of C57 (wild-type) and P23H mice after 2 weeks of treatment with 4-PBA or vehicle-only (CTL). d Quantification of the western blots represented by c. e Transcript levels of xbp1s in retinas of P23H mice treated with 4-PBA or vehicle-only (CTL) for 2 weeks, normalized to aged-matched C57 mice. Statistical analyses with one-way ANOVA. Results are shown as individual symbols representing data values along with mean and standard deviation (SD). *p ≤ 0.05. **p ≤ 0.01. ***p ≤ 0.001. ****p ≤ 0.0001