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BACE1-cleavage of Sez6 and Sez6L is elevated in Niemann-Pick type C disease mouse brains

Fig 7

Sez6L immunoreactivity is lost in Purkinje neurons upon their neurodegeneration in 10-weeks old NPC1 vs. wt mouse brains.

Sez6L (green) is specifically expressed in neurons of the cornu ammonis (CA) and granular layer of dentate gyrus (DG) of the hippocampus, as well as in deep layer cortical neurons and Purkinje cells in the cerebellum in 10-weeks old wt mice. Due to profound neurodegeneration, 10-weeks old NPC1 mice show a substantial loss of Sez6L intensity in Purkinje neurons. DAPI (blue) was used to counterstain all nuclei. ca1, cornu ammonis 1; mo, molecular layer; gr, granular layer.

Fig 7

doi: https://doi.org/10.1371/journal.pone.0200344.g007