Figure 6.

Arimoclomol treatment potentiates upregulation of the UPR in P23H-1 rats. (a–c) Representative western blots of retinal lysates from wild-type or P23H-1 rats at P35 for antibodies against the three branches of the UPR: (a) the PERK branch (phosphorylated eIF2α, ATF4 (arrow indicates specific ATF4 band) and GADD34); (b) the ATF6 branch (cleaved or nuclear (N) ATF6 and full-length (FL) ATF6); and (c) the IRE1α branch (phosphorylated IRE1α). Actin was used as a loading control throughout. (d) RT-PCR using primers targeting spliced XBP1 and unspliced XBP1 in cDNA from P23H-1 retinae at 5 weeks. Rpl19 was used as a normalisation control. (e) Quantification of RT-PCR using primers targeting spliced XBP1 and unspliced XBP1 in cDNA from P23H-1 rats at P35. The ratio of spliced XBP1 to unspliced XBP1 was normalised to Rpl19 levels and normalised to wild-type XBP1 ratio. Statistical significance was determined by using analysis of variance, *P<0.05, ***P<0.001