In vivo demonstration
of optimized B4galnt2 gene activation. (a)
Representative slide scan images of liver sections showing RNAscope
staining for B4galnt2 mRNA (green) and DAPI (blue). Insets depict
the relative locations of 4× and 16× views. Scale bars are
800 μm for 1×, 200 μm for 4×, and 50 μm
for 16× images. Time courses of B4galnt2 mRNA (b) and activator
mRNA (c) copy numbers from liver tissue over 9 days. B4galnt2 mRNA
copy numbers (d) and percentage of activated hepatocytes (e) in mice
treated with activator mRNA and B4 sgRNA with or without AcrIIA4 co-delivery.
The “–AcrIIA4” groups were dosed with activator
mRNA and B4 sgRNA on day 0. The “+AcrIIA4” groups were
simultaneously dosed with activator mRNA, B4 sgRNA, and AcrIIA4 mRNA
on day 0. All VPR and VPH-SS18-treated mice were euthanized at day
1 postinjection, and all p300-treated mice were euthanized at 5 days
postinjection. (f) VPH-SS18 time course with redosing. VPH-SS18 mRNA
and B4 sgRNAs were delivered to both groups on day 0. AcrIIA4 treatment
was given to one group on day 5, followed by euthanasia on day 6.
Remaining mice that did not receive AcrIIA4 mRNA were then redosed
with VPH-SS18 mRNA and B4 sgRNAs on day 14. Data represent mean ±
SEM (n = 3–4 mice). Statistical significance
was assessed using a two-way ANOVA followed by Dunnett’s multiple
comparison compared to the NT-treated group (b,c) and a student’s t test (d,e) (*P < 0.05, **P < 0.01, ***P < 0.001, ****P < 0.0001).