Figure 5.

Storage in the CNS of post-symptomatic AAV-treated SD cats. (a) Storage in untreated SD cats is visualized by dark PAS staining in gray matter. In treated brains, ganglioside storage was present in Hex deficient areas (see corresponding naphthol staining for the same cat, 11-831, in Figures 4b and c), such as the temporal lobe (blocks d and e) and cervical spinal cord (block k). (b) Sample sites for sialic acid quantitation (circles) in brain (A-H) and spinal cord (K and O; half of each block was used). (c) Sialic acid levels were measured in untreated SD cats (n = 4) and in the EPS (n = 4) and LPS (n = 3) cohorts for comparison to normal cats (n = 4 for each cohort). **, all samples from untreated cats were significantly higher than normal (P = 0.015 for each block) in the cerebrum (2.6 – 4.9 fold normal), brainstem and cerebellum (2.0 - 3.8 fold normal) and spinal cord (3.2 - 3.8 fold normal); *, samples from treated cats that were not significantly higher than normal. Samples without this symbol were significantly higher than normal (P ≤ 0.030 for each block); ▼, samples from treated cats that were significantly lower than untreated (P ≤ 0.030); ▲, sample from treated cats that was significantly higher than untreated (P = 0.015); ●, sample that was significantly lower in the LPS cohort versus the EPS cohort (P = 0.037). Abbreviations: EPS = early post-symptomatic; LPS = late post-symptomatic; tx = treatment. Data is expressed as mean ± s.d. Samples were analyzed in duplicate and the average value is reported.