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. 2023 May 24;618(7964):402–410. doi: 10.1038/s41586-023-06090-9

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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 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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Extended Data Fig. 1 — a) The body weight of KO mice is reduced (2 months: 15 WT and 15 KO mice; 22 months: 9 WT and 11 KO mice; 2-sided unpaired Student’s t-test: p = 0.0034). b) Cortical neurons are decreased in aged KO mice. Cross sections of the motor cortex of 22-month-old WT and KO mice with NeuN-labelled neurons (1 exp.; 3 mice per genotype; 2-sided unpaired Student’s t-test: p = 0.0312). Scale bars: 50 µm. c) Progressive Purkinje cell loss in KO mice. Representative images of the Purkinje cell layer of HE-stained sagittal cerebellum sections of 22-month-old WT and KO mice (1 exp.; n = 3 mice with 3 sections each per genotype; 2-sided unpaired Student’s t-test: WT 2 versus WT 22 months, p = 0.0002; KO 2 versus KO 22 months p = 0.0001, KO versus WT 22 months p = 0.0001). Scale bars: 100 µm. d) Swollen corticospinal axon in the lumbar spinal cord of a 6-month-old KO mouse (1 exp.). TEM of a horizontal spinal cord section. Scale bar: 5 µm. The higher magnification shows the accumulation of granular electron-dense material, defective organelles and tubulofilamentous material. The axon is surrounded by a distended myelin sheath (arrowheads). Scale bar: 1 µm. e) Progressive α-motoneuron loss in the thoracic spinal cord of KO mice. Nissl-stained horizontal sections (1 exp.; 3 mice per genotype; 2-sided unpaired Student’s t-test: WT 22 versus KO 22 months p = 0.0322, KO 2 versus KO 22 months p = 0.0018, WT 2 versus WT 22 months p = 0.0154). f) The Musculus gastrocnemius mass is reduced in 2-month-old KO mice (1 exp.; 3 WT and 3 KO mice with 2 samples each (left and right); 2-sided unpaired Student’s t-test: p = 0.001). g) Grouped atrophic skeletal muscle fibres in 20-week-old KO mice. Toluidine-blue stained semi-thin sections (1 exp.). Scale bar: 100 µm. h) Fragmentation and innervation loss of neuromuscular junctions (NMJ) in the Musculus gastrocnemius of 2-month-old KO mice stained with α-bungarotoxin (red), neurofilament 200 (green) and Hoechst 33258 (1 exp.; 3 mice per genotype). 2-sided unpaired Student’s t-test for fragmentation (p = 0.0012). i) The abundance of some ER proteins with RHDs is changed in brain lysates of 5-month-old KO mice (1 exp. with 5 samples per genotype; 2-sided Mann-Whitney-U-test: Rtn2a+b p = 0.0317, Rtn4a p = 0.0317, Fam134b p = 0.0079, Reep2 p = 0.0159). Quantitative data are shown as mean ± SEM.

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