The chymotrypsin-like protease activity was quantified in the ROs after 4 weeks-several doses of PMO treatment (n = 20 ROs per treated group per biological replicate) using the Proteasome Activity Assay Kit (Abcam) following manufacturer’s protocol. Protease activity was measured by the fluorescence intensity (Excitation/Emission = 350/440 nm) using a Varioskan LUX Multimode Microplate Reader and normalized to the protein amount.
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