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. 2022 Apr 25;10:60. doi: 10.1186/s40478-022-01355-2

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© The Author(s) 2022

Open AccessThis 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/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

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Fig. 1 — Premature death, absence of dystrophin expression and muscle atrophy in R-DMDdel52 rats. a Scheme of CRISPR-mediated gene deletion in rats and genotyping data from WT and R-DMDdel52 animals. Position of WT and deletion (del52) bands is indicated. b Pictures of R-DMDdel52 rats (left) and a WT littermate (right) aged 12 months, showing a marked facial muscle atrophy that modifies the appearance of the animal. c Kaplan Meier Curve for the frequency of WT (black curve) and R-DMDdel52 (red curve) rat survival. d Representative immunofluorescence for DYSTROPHIN (orange) and ßDYSTROGLYCAN (red) in tibialis anterior sections of 3-month-old (left panels) and 12-month-old (right panels) WT and R-DMDdel52 rats. Scale bar 20 μm. e Quantification of the percentage of dystrophin-positive fibres in WT and R-DMDdel52 TA at 3 weeks, 3, 6, 12 months of age. f Western blot analysis of WT and R-DMDdel52 proteins extracted from soleus and diaphragm tissues. The amount of loaded proteins was normalized on β-TUBULIN levels. g CK levels of WT and R-DMDdel52 rats aged 3 weeks, 3 months, 6 months and 12 months. h Weight curve showing that R-DMDdel52 rats exhibited a progressive loss of weight compared to their healthy littermates (WT). i) Graph showing the body and tibial length in centimetres of R-DMDdel52 and WT rats. j) Pictures of R-DMDdel52 muscles at 12 months of age: tibialis anterior, extensor digitorum longus, soleus, and heart. k) Graph showing the weight in grams of different skeletal muscles from R-DMDdel52 rats and WT aged 12 months