2020
DOI: 10.1113/jp279282
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Desmin prevents muscle wasting, exaggerated weakness and fragility, and fatigue in dystrophic mdx mouse

Abstract: Key points Desmin, similar to dystrophin, is associated with costameric structures bridging sarcomeres to the extracellular matrix. Deletion of the desmin gene in mdx mice [double knockout (DKO) mice] induces marked muscle weakness and fatigue resistance compared to mdx mice. Muscle fragility (higher susceptibility to contraction‐induced injury) was also aggravated in DKO mice compared to mdx mice. By contrast to mdx mice, the DKO mice did not undergo muscle hypertrophy. Desmin cDNA transfer with adeno‐associ… Show more

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Cited by 19 publications
(54 citation statements)
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References 61 publications
(173 reference statements)
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“…Although various studies have investigated the role of dystrophin in DMD, little is known about the therapeutic potential of targeting intermediate filaments, which have an important role in linking sarcomeres to the extracellular matrix. The characterization of desmin as a potential therapeutic target that could attenuate muscle wasting in a DMD model is an important implication reported by Ferry et al (2020) in The Journal of Physiology. In their study, Ferry et al (2020) provide experimental evidence indicating that desmin, a muscle-specific, type III intermediate filament, may play important roles in locomotor muscle performance, fragility and wasting in dystrophic mdx mice.…”
mentioning
confidence: 98%
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“…Although various studies have investigated the role of dystrophin in DMD, little is known about the therapeutic potential of targeting intermediate filaments, which have an important role in linking sarcomeres to the extracellular matrix. The characterization of desmin as a potential therapeutic target that could attenuate muscle wasting in a DMD model is an important implication reported by Ferry et al (2020) in The Journal of Physiology. In their study, Ferry et al (2020) provide experimental evidence indicating that desmin, a muscle-specific, type III intermediate filament, may play important roles in locomotor muscle performance, fragility and wasting in dystrophic mdx mice.…”
mentioning
confidence: 98%
“…The characterization of desmin as a potential therapeutic target that could attenuate muscle wasting in a DMD model is an important implication reported by Ferry et al (2020) in The Journal of Physiology. In their study, Ferry et al (2020) provide experimental evidence indicating that desmin, a muscle-specific, type III intermediate filament, may play important roles in locomotor muscle performance, fragility and wasting in dystrophic mdx mice. Desmin is integral to the contractile mechanism because it connects myofibrils at the Z-disk level, as well as the Z-disk to the sarcolemma.…”
mentioning
confidence: 98%
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