CHKA and Enlarged kidney: DS-4108b led to improvements in blood urea nitrogen (BUN) levels, plasma creatinine concentrations, and renal glycogen concentrations of cKI-Mut mice (Supplemental Figure 3, G–I), but we observed no clear correction of the aberrant splicing in kidney, the nephromegaly, or the abnormal renal histology in cKI-Mut mice (Supplemental Figure 3, J–L).