Percentage fractions of (β-Gal+) PCs in 32-week-old heterozygous females differ between cerebellar lobules affected and spared from degeneration of Purkinje.

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Percentage fractions of (β-Gal+) PCs in 32-week-old heterozygous females differ between cerebellar lobules affected and spared from degeneration of Purkinje cells. Percentage fractions of (β-Gal+) PCs in 32-week-old heterozygous females differ between cerebellar lobules affected and spared from degeneration of Purkinje cells. (A) Similar to lobule X, PCs that remain in lobule I-II uniformly express β-Gal (white arrowheads) in mutant males. Small β-Gal+ non-PC nuclei largely correspond to the population of (Bergmann glia) astrocytes (Fig. S1A). Axons of these remaining PCs in lobule I-II develop spheroids (blue arrowhead in the inset). (B,C) β-Gal expression in PCs is mosaic (white arrowheads) in heterozygous females and varies among individual animals. The fraction of β-Gal+ PCs is lower in lobule I-II compared with lobule X. β-Gal expression is also mosaic in other cell types within the PC layer (Bergmann glia astrocytes; Fig. S1B). (C) β-Gal+ PCs in heterozygous females also develop spheroids on their axons (blue arrowhead in the inset). (D) The percentage fraction of β-Gal+ PCs in lobule I-II (n=17) is significantly lower than in lobule X (n=14) of heterozygous females. *P<0.0001. Images shown for lobule I-II and X in A,B were collected from cerebella of a single mutant male and heterozygous female, respectively. Lobule I-II PC densities and percentages of β-Gal+ PCs in A-C represent values for the particular animals shown. The z-thickness of MIP projections in A-C is 18-22 µm. Scale bars: 50 µm. Jakub Sikora et al. Dis. Model. Mech. 2016;9:13-23 © 2016. Published by The Company of Biologists Ltd