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NUBPL and FOXRED1 cDNA rescue of CI defects in patient fibroblasts. Barplots show CI activity, normalized by CIV activity, measured in control and patient fibroblasts, before and after transduction with wild-type NUBPL-V5 mRNA (a) or wild-type FOXRED1-V5 mRNA (b). Bars show mean of 3 biological replicates, and error bars indicate ±1 s.e.m. Asterisks indicate p<0.01. Representative dipstick assays shown below.
