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. 2020 Sep 4;8:154. doi: 10.1186/s40478-020-01001-9

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© The Author(s) 2020

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 — FLNc expression in different mouse muscles, and soleus muscle of wildtype and Hom mice. a Protein quantification in different wildtype mouse muscles demonstrated highest FLNc expression levels in soleus muscle. Anti-GAPDH was used as loading and normalization control. n = 5. EDL: Musculus (M.) extensor digitorum longus; Gast: M. gastrocnemius; Quad: M. quadriceps; TA: M. tibialis anterior; Sol: M. soleus; Dia: diaphragm. b Quantitative real time PCR showed virtually identical total Flnc mRNA levels in adult WT and Hom animals (n = 4–6). c Comparison of Flna and Flnb mRNA expressions using the Biorad CFX manager software version 3.1 and Student’s t-test revealed no significant differences in Hom animals (n = 4–6). d, e Western blotting of total soleus muscle extracts from adult mice using an antibody recognizing both, WT and mutant FLNc (FLNc total) in conjunction with protein quantification using a LI-COR Odyssey Infrared Imaging System. Statistical analysis using Mann-Whitney U-testing indicated a significant reduction to approximately 40% of total FLNc expression in Hom animals. Results are shown as mean +/− IQR (n = 4; p = 0.0286). Staining with an antiserum recognizing the carboxy-terminal 16 amino acids of FLNc (and thus only WT FLNc) confirmed its complete absence in Hom animals (n = 3)