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. 2021 Sep 2;11:17557. doi: 10.1038/s41598-021-96181-2

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© The Author(s) 2021

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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Over-expression of PDI is protective against mutant FUS induced Golgi fragmentation in a neuronal cell line. (A) Neuro-2a cells co-expressing EGFP (row 1), FUS-GFP-WT (row 2) or mutant FUS-GFP-R521G with either empty vector, (row 3) or PDI (row 4) at 72 h post-transfection, were subjected to immunocytochemistry for Golgi marker GM130 and counter-staining with Hoechst 33342 stain (nuclei). Most cells expressing EGFP, or FUS-GFP-WT (row 1 and 2) contained an intact Golgi apparatus. Conversely, cells expressing mutant FUS-GFP-R521G with empty vector displayed fragmented Golgi, indicated with white arrows (row 3). However, over-expression of PDI reduced Golgi fragmentation in these cells (row 4). Scale bar = 5 µm. (B) Quantification of cells in (A). A significant reduction (*p < 0.05) in the proportion of cells with Golgi fragmentation was observed in cells expressing mutant FUS-GFP-R521G with PDI, compared to those expressing mutant FUS with vector only. Values represent mean ± SD, n = 3. *p < 0.05.