Figure 5.

Reduction in Accumulation of GAGs by JR-141 in the Brain and the Peripheral Tissues of TFRC-KI/Ids-KO Double-Mutant Mice

JR-141 was intravenously administered to the mice at a dose of 1, 3, or 10 mg/kg BW once a week for 4 weeks. GAGs were quantified 1 week after the final dosing in the resected brain, heart, kidney, liver, lung, and spleen. Values are expressed as % accumulation when mean GAG levels in the WT mice were considered to be 0% and those in Ids-KO mice were considered to be 100%. Bars indicate the mean for each group (n = 4). **p < 0.01 by Dunnett test (Ids-KO versus JR-141 groups) and ^##p < 0.01 by Student’s t test (Ids-KO versus hIDS groups). N.S., not significant. The absolute values of the concentration and detailed statistical analysis between groups are presented in Table S3.