ArticleNeuropathology and applied neurobiology2025
Quantification of Exercise-Induced Sarcomeric Damage in R349P Desmin Knock-In Mice: A New Approach in Myofibrillar Myopathy Research.
Article in Neuropathology and applied neurobiology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.
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2 citing papers in PubMed.
- Identifying regulatory muscle processes in infantile and adult Pompe patients using a multi-omic and morphological approach.Brain pathology (Zurich, Switzerland) · 2026Article
- Quantification of Exercise-Induced Sarcomeric Damage in R349P Desmin Knock-In Mice: A New Approach in Myofibrillar Myopathy Research.Neuropathology and applied neurobiology · 2025Article
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13 authors.
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Abstract
aimsThe classical morphological hallmarks of the clinically and genetically diverse group of human myofibrillar myopathies are signs of myofibrillar degeneration and desmin-positive protein aggregates. The local sarcomeric enrichment of filamin-C and xin actin-binding repeat-containing proteins 1 and 2 (xirp 1 and xirp 2) is a marker of myofibrillar damage. This work aimed to (i) address filamin-C- and xirp 1/2-positive sarcomeric lesions in desminopathy mouse models, (ii) develop an approach to quantifying xirp 1/2-positive sarcomeric lesions, and (iii) study the effects of acute physical exercise on sarcomeric lesion formation in R349P desmin knock-in mice, which are a model of human R350P desminopathy.
methodsSarcomeric lesions were visualised by xirp 1/2 and filamin-C immunofluorescence in soleus muscles from R349P and R405W desmin knock-in, desmin knock-out and W2711X filamin-C knock-in mice. The open-source software QuPath was used to analyse xirp 1/2 confocal immunofluorescence images of soleus muscle from nonexercised and treadmill-exercised R349P desmin knock-in mice.
resultsFilamin-C and xirp 1/2 stained muscles revealed the presence of congenerous sarcomeric lesions in heterozygous and homozygous R349P and R405W desmin knock-in, homozygous desmin knock-out and heterozygous and homozygous W2711X filamin-C knock-in mice. Quantitative analysis of R349P desmin knock-in mice showed (i) significantly more lesions in nonexercised heterozygous mice, with an even more pronounced increase in homozygous animals, as compared to wild-type, and (ii) a significant increase in sarcomeric lesions per mm
conclusionsA QuPath workflow to quantify sarcomeric lesions using xirp 1/2 immunofluorescence images showed augmented densities of lesions in R349P desminopathy mice and after treadmill exercise. Eccentric and high-intensity physical activity may exhibit a disease-promoting effect on skeletal muscles in desminopathy.
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