Transgenic Rescue of the LARGEmyd Mouse: A LARGE Therapeutic Window?
Fig 7
Levels of transgenic LARGE transcript are equivalent to endogenous expression in brain, but greatly overexpressed in heart and skeletal muscle.
qPCR analysis of cDNA prepared from brain, heart and quadriceps (from genotypes as indicated) using primers for endogenous LARGE (a) or the LARGE-LV5 transgene (b). Quantities are shown as mean number of transcripts per ng cDNA (+ S.E.M, N = 5–9). Dashed line in (b): expression level of endogenous LARGE in brain. ** P<0.01, *** P<0.005, **** P<0.001. No significant differences in endogenous expression within a given tissue were detected between WT and WT-LV5, or in transgene expression between WT-LV5 and LARGEmyd-LV5, for any of the tissues studied.
