Severe kidney dysfunction in sialidosis mice reveals an essential role for neuraminidase 1 in reabsorption.

Activities of nontargeted lysosomal glycosidases (β-hexosaminidases A and B, and β-galactosidase) showed a significant increase in all tissues of Neu1 ΔEx3 mice except for the liver, where total β-hexosaminidase activity showed a trend for an increase ( Figure 2, C and D ).

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In contrast, labeling with RCA-1, usually exposed in complex glycans, after neuraminidase treatment, was reduced, while PNA labeling showed a nonsignificant trend toward reduction ( Figure 5, A and B ).

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