Figure 4.

GNeo-IDUA Reduces Histopathological Hallmarks in the Aged MPSI Mouse Brain

Idua^−/− mice were treated intranasally with 1 mg/kg IDUA or GNeo-IDUA twice a day for 30 days and then once a day for 30 days. Coronal sections were taken from different brain regions and stained with antibodies against LAMP1, a lysosomal marker, or GFAP, a marker of astrocytosis. (A) Representative confocal images acquired from olfactory bulbs. (B) Quantification of LAMP1 and (C) GFAP in the granule cell layer of the olfactory bulb. (D) Representative confocal images acquired from the primary somatosensory cortex. (E) Quantification of LAMP1 and (F) GFAP in layer II/III of the primary somatosensory cortex. n = 3 animals per treatment group. Error bars indicate SEM, and significance was determined by one-way analysis of variance (ANOVA) with Tukey’s post-hoc analysis.