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. 2020 Feb 26;10(3):355. doi: 10.3390/biom10030355

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© 2020 by the authors.

Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).

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Representation of the main proteomic modifications induced by NAGLU deletion in murine brains. Dysregulation of cytoskeleton associated proteins and the energy metabolism-involved proteins may contribute to impair the lysosomal membrane trafficking pathway linked to the pathogenesis of neuropathy in MPS IIIB disease. Both altered pathways are relevant to preserve brain homeostasis and could be responsible for an impairment of synaptic vesicle formation and activity in the MPS IIIB mouse brain.