Figure 5. NMDI-1 co-administration with gentamicin alleviates secondary MPS I-H lysosomal markers.

Idua ^W392X mice were administered gentamicin (g) or NB84 (n) for 14 days without (−) or with (+) co-administration of NMDI-1 during the final 3 days of treatment. β-hexosaminidase (β-hex) and β-glucuronidase (β-gluc) enzyme activities were determined in tissue lysates from A) brain and B) spleen. The data are expressed as the fold-elevation in enzyme specific activity measured in treated and untreated Idua ^W392X mice relative to the level in WT mice, which was normalized to 1. The data are the mean +/− sd of the values obtained from 3–4 mice per treatment group performed with ≥8 replicates (n =  3 or 4). p values above the brackets compare mice administered NMDI-1 versus those without NMDI-1 administration within each treatment group.