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. 2013 Apr 10;8(4):e60478. doi: 10.1371/journal.pone.0060478

Figure 6. Improvements in MPS I-H biochemical markers are sustained in Idua W392X mice co-administered gentamicin and NMDI-1 for 9-weeks.

Figure 6

Idua W392X mice were administered gentamicin 3-times weekly for 9 weeks without (−) or with (+) co-administration of NMDI-1 or vehicle alone (v) two times weekly. MPS I-H biochemical markers were analyzed in brain tissue from treated Idua W392X mice and controls. A) α-L-iduronidase (Idua) specific activity was determined in brain protein lysates. The data are expressed as the specific activity in the mutant mouse tissues relative to WT controls × 100 (% WT Idua Activity). Data are the mean +/− sd of values obtained from 5 mice per group, performed using ≥4 replicates (n = 5). p values above the columns compare treated mice to untreated controls, while the p values above the brackets compare mice treated with NMDI-1 to those without NMDI-1 treatment. B) Sulfated GAGs were quantitated in defatted, dried brain homogenates from WT mice (light gray bars) and from untreated and treated Idua W392X mice (dark gray bars). GAG levels were quantitated as micrograms GAGs per milligram protein. The dashed line represents the WT GAG level as a reference. Data are expressed as mean +/− sd of 15–18 assays from 5 mice for each experimental group (n = 5). p values above the columns compare treated versus untreated W392X mice. p values above the brackets compare mice co-treated with both gentamicin and NMDI-1 compared to mice treated with gentamicin alone. C) β-hexosaminidase (β-hex) and β-glucuronidase (β-gluc) enzyme activities were determined in brain protein lysates. 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 5 mice per treatment group performed with ≥4 replicates (n = 5). p values above the brackets compare mice administered NMDI-1 versus those without NMDI-1 administration within each treatment group.