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View full-text article in PMC

. 2020 Feb 1;9(2):396. doi: 10.3390/jcm9020396

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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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Impaired autophagic flux and accumulation of misfolded proteins in brain cortex tissues of human MPS patients. Representative confocal microscopy images showing (A) accumulation of light chain 3B protein (LC3B)-positive puncta (green) and (B) thioflavin S-positive protein aggregates (green) in brain cortex tissues from MPS I (561), MPS II (902), MPSIIIA (3617 and 563), MPS IIIC (6194), and MPS IIID (5411 and 5424) patients and a representative control (662). The fixed brain tissues of the MPS II patient HBCB1801OC were not sufficiently preserved to perform cryosectioning and conduct immunofluorescent analysis. DAPI (blue) was used as the nuclear counterstain. Neurons with LC3B puncta are marked with arrowheads and those stained with thioflavin S are markedwith asterisks. Scale bars: 20 µm (A) and 50 µm (B).