Neuraminidase 1 secondary deficiency contributes to CNS pathology in neurological mucopolysaccharidoses via brain protein hypersialylation.

Injection of LV-CTSA-IRES-NEU1-GFP did not alter levels of the synaptic proteins in WT mice but led to a significant increase of VGLUT1 and PSD-95 levels in Hgsnat P304L mice, whereas the levels of Syn1 showed a nonsignificant trend toward an increase ( Figure 10 ).

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The combined activities of lysosomal β-hexosaminidases A and B were significantly increased in all patient samples except for MPS II, in which they showed a trend toward an increase ( Figure 1A ).

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