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. Author manuscript; available in PMC: 2012 Apr 1.
Published in final edited form as: Mol Genet Metab. 2010 Dec 31;102(4):436–447. doi: 10.1016/j.ymgme.2010.12.014

Figure 1. Pathology in 9H/PS-NA brain.

Figure 1

Brain sections were from 20-wk 9H/PS-NA mice. Upper panels: H&E staining sections showed (A) Normal neuron cells in WT and (B) axonal spheroids (arrows) suggesting degenerative neurons in cerebral cortex of 9H/PS-NA mice. Lower panels: Electron micrographs of neurons in 9H/PS-NA and WT deep cortical layer. (C) Neuron in heterozygote littermate. (D) Degenerating neurons in 9H/PS-NA mice showed irregular nuclear membranes with condensed chromatin (n), multiple mitochondria (m) and filament structures, and electronic dense particles (arrows) in the cytoplasm. (E) The axons in 9H/PS-NA brain showed accumulations of complex electron dense inclusion bodies (arrows) that were amorphous. The myelin sheaths of axons appeared normal in 9H/PS-NA mice. The scale bars are shown in each image. n=nuclei.