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. 2015 Aug 18;11(8):e1005454. doi: 10.1371/journal.pgen.1005454

Fig 3. Loss of Spatacsin causes severe neuron loss in the motor cortex and the cerebellum.

Fig 3

(A-B) Brain size is reduced in 16-month-old KO compared to WT mice. Scale bars: 2 mm. (C) Progressive reduction of brain weight in KO mice (n = 3; Student’s t-test: *** indicates p<0.001). (D-F) Neuron loss in the motor cortex of 16-month-old KO mice. Hoechst-33258 (blue; nuclei), NeuN (red; neuronal marker), and Ctip2 (red; marker for layer V neurons) staining of the motor cortex at 16 months of age from WT (D) and KO (E) mice. Individual cortical layers are labeled (I–VI). Scale bars: 100 μm. Quantification of NeuN-positive cells reveals a depletion of neurons from layers V-VI but not layers I-III of the motor cortex at 16 months of age in the KO (n = 3; Student’s t-test: *** indicates p<0.001). (G, H) Nissl stainings of sagittal brain sections do not support a thinning of the corpus callosum of 16-month-old KO mice. Scale bars: 500 μm (I-K) Semithin sections of the lumbar corticospinal tract show a loss of large diameter axons in 8- and 16-month-old KO mice (n = 3; Student’s t-test: *** indicates p<0.001). Scale bars: 20 μm. (L-N) Purkinje-cell loss in aged KO (M) but not WT (L) mice. ML: Molecular layer; PCL: Purkinje cell layer; GL: granular layer. The somata of Purkinje cells is indicated by a dotted line. (N) Quantification of Purkinje cells in hematoxylin-eosin stained cerebellum sections indicates a severe loss of Purkinje cells in aged KO mice (n = 3; Student’s t-test: *** indicates p<0.001). Scale bars: 40 μm.