This is the authors' abstract. We don't add key points for this paper.
BACKGROUND: Desminopathy is a progressive myopathy and the most common intermediate filament disease in humans. Desmin is an essential part of the filamentous network that aligns myofibrils, anchors nuclei and mitochondria and connects the z discs to the sarcolemma. We challenged a rat model with a mutation in R349P Des, analogous to the most frequent R350P DES missense mutation in humans, through eccentric-biassed exercise training. METHODS: R349P Des rats (Des) and their wild type (WT) and heterozygous (HET) littermates were subjected to 4 weeks of downhill running. At the end of 4 weeks, muscles were collected and assessed histologically and biochemically. RESULTS: Des rats demonstrated significantly lower running capacity compared to wild type and heterozygous littermates (p < 0.0001), which exacerbated over the course of the study (p < 0.001). Running performance in Des rats decreased by ~50% after 4 weeks of downhill running. Desmin-positive skeletal muscle aggregates, a histopathological hallmark of desminopathy, were ~2-fold higher after downhill running in the Des group (p < 0.05). On a protein level, we found a differential exercise response of Des compared to WT affecting various cellular pathways concerning autophagy, proteasomal activity, energy metabolism and cytoskeletal proteins. CONCLUSIONS: Our results suggest an impaired ability of Des muscle to adapt to chronic eccentric-biassed exercise and a subsequent exacerbation of the functional and histopathological phenotype.
Answers are grounded in this paper and cite their sources.
No takes yet. Share an insight, caveat, or question.
Langer et al. (2026) studied this question.
Synapse has enriched 10 related papers. Consider them for comparative context: