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. 2023 Feb 8;14:692. doi: 10.1038/s41467-023-36096-w

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© The Author(s) 2023

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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Fig. 9 — AI-based quantitation of neuronal tomograms containing mitochondria demonstrates a significant increase in mitochondrial granule size with higher polyQ in HD human and mouse model neurons. While this is rescued by PIAS1 hetKO and GRSF1 siRNA in iPSC neurons, it is only partially rescued by Pias1 KD in mouse model neurons. Violin plots displaying AI measurements of mitochondrial granule a volume (Kruskal Wallis statistic = 857.2, P value < 0.0001 with Dunn’s multiple comparisons) and b numbers per nm³ of mitochondrial volume (Kruskal Wallis statistic = 129.6 P < 0.0001 with Dunn’s multiple comparisons) from cryoET tomograms of neurites for several HD patient iPSC-neuronal cell lines (number of tomograms per cell line: Q18 = 21, Q20 = 20, Q53 = 14, Q66 = 10, Q77 = 5, Q109 = 37, Q53 PIAS1 hetKO = 22, Q66 PIAS1 hetKO = 68 and Q66 GRSF1 siRNA = 17; number of granules per cell line:Q18 = 364, Q20 = 774 Q53 = 254, Q66 = 249, Q77 = 94, Q109 = 1534, Q53 PIAS1 hetKO = 13, and Q66 PIAS1 hetKO = 2524 and Q66 GRSF1 siRNA = 958) as well as mitochondrial granule c volume (Kruskal Wallis statistic = 1030, P < 0.0001 with Dunn’s multiple comparisons) and d numbers per nm³ of mitochondrial volume (Kruskal Wallis statistic = 68.28, P < 0.0001 with Dunn’s multiple comparisons) from cryoET tomograms of neurites for three mouse neuron models (number of tomograms per mouse model: WT = 31, BACHD = 22, dN17 BACHD = 15, BACHD Control siRNA=5 and BACHD Pias1 siRNA=12; number of granules per mouse model:WT = 2057, BACHD = 634, dN17-BACHD = 477, BACHD Control siRNA = 91 and BACHD Pias1 siRNA = 92). The human neurons and mouse model neurons showed an increase in granule volumes in all but Q109, a trend of reduced granule number in human neurons and a significant reduction in granule number in mouse model neurons. Median displayed as dashed line, dotted lines display quartiles. ns = not significant, ****p < 0.0001, **p < 0.01, *p < 0.05 For full statistical details refer to Supplementary Table 2. Source data are provided as a Source Data file.