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. 2020 Nov 23;10:20365. doi: 10.1038/s41598-020-77032-y

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© The Author(s) 2020

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 licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence 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 licence, visit http://creativecommons.org/licenses/by/4.0/.

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Enlargement of the lysosomal compartment in the cerebellum of MPS IIIB dogs. (A) Immunostaining for the lysosomal marker LAMP1 provides a surrogate marker for storage material accumulation and reveals a progressive increase in the size of the lysosome in the cerebellum of MPS IIIB dogs (MPS IIIB—grey) compared to unaffected controls (unaffected—white). These changes, examined using thresholding analysis, were apparent in the molecular layer, the Purkinje cell layer, the cerebellar white matter and the deep cerebellar nuclei (DCN). (B–E) Thresholding image analysis of LAMP1 immunostaining confirms the progressive increase in staining intensity for this marker in the cerebellum of MPS IIIB dogs compared to unaffected controls. Markedly increased LAMP1 staining was evident in all regions except the white matter (D) by 15 months of age, and in all regions by later stages (~ 28 months). Error bars indicate SEM. Number of animals analyzed (3 mo: unaffected n = 2, MPS IIIB n = 2; 9 mo: unaffected n = 2, MPS IIIB n = 2; 15 mo: unaffected n = 2, MPS IIIB n = 3; > 20 mo: unaffected n = 2, MPS IIIB n = 3). Numbers next to ages indicate animal ID number.