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. 2019 Jul 9;9:9891. doi: 10.1038/s41598-019-45859-9

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© The Author(s) 2019

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/.

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Selective pattern of neurodegeneration is observed in ovine model of INCL. (A) Gross anatomical disparity identified between WT control (left) and PPT1 homozygote (right) brains. Total fresh brain weight is significantly reduced in homozygote sheep shown in scatter plot (P = 0.0006). (B) Comparison of orthogonal T2-weighted spin echo imaging slices in example wild type (top row) and homozygous (bottom row) animals. There is global reduction in cerebral volume (red arrows), with ventriculomegaly (blue arrows) and thinning of the frontal cortex in the homozygote sheep. Note increased fluid signal between cerebellar folia (green arrows) suggestive of neocerebellar atrophy. (C) Volumetric renderings showing regions of interest for bilateral motor cortex (red), cerebellum (green), and tri-ventricular cerebrospinal fluid (blue). (D) Scatter plot with bar chart of volumetric data for the bilateral motor cortex, triventricular CSF and cerebellum with corresponding 3D images above each data point relating to the area and genotype. Statistical analyses utilized unpaired two-tailed Student’s t-test. Error bars represent SEM.