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Figure 4 | Journal of Neurodevelopmental Disorders

Figure 4

From: Mouse models of the fragile X premutation and fragile X-associated tremor/ataxia syndrome

Figure 4

Ectopic expression of a CGG90 repeat results in Purkinje cell loss. (A) Cerebellum of control mouse without a CGG90 repeat (that is, L7Fmr1) showing normal distribution of Purkinje cells in the Purkinje cell layer. (B) Higher magnification of the Purkinje cell layer in control mouse. (C) Selective Purkinje cell loss in 32-week-old mouse expressing a CGG90 repeat under the L7 Purkinje cell-specific promoter (that is, L7CGG90Fmr1). (D) Purkinje cell loss in shown at higher magnification in L7CGG90Fmr1 mouse. gl, granule cell layer; ml, molecular layer; pcl, Purkinje cell layer. (Adapted from [34]).

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