ArticleBiophysical journal2010
Microarchitecture is severely compromised but motor protein function is preserved in dystrophic mdx skeletal muscle.
Article in Biophysical journal, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 42 papers.
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Who cites it
42 citing papers in PubMed, 80 citations in OpenAlex.
- 'Cells · 2026Article
- Twisting Paths: The Paradox of Fiber Branching in Muscle Regeneration.International journal of molecular sciences · 2026Review
- Acute microtubule changes linked to DMD pathology are insufficient to impair contractile function or enhance contraction-induced injury in healthy muscle.bioRxiv : the preprint server for biology · 2024Article
- MYH10 activation rescues contractile defects in arrhythmogenic cardiomyopathy (ACM).Nature communications · 2023Article
- SEMPAI: a Self-Enhancing Multi-Photon Artificial Intelligence for Prior-Informed Assessment of Muscle Function and Pathology.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2023Article
- Effect of tissue fixation on the optical properties of structural components assessed by non-linear microscopy imaging.Biomedical optics express · 2023Article
- Single fibre cytoarchitecture in ventilator-induced diaphragm dysfunction (VIDD) assessed by quantitative morphometry second harmonic generation imaging: Positive effects of BGP-15 chaperone co-inducer and VBP-15 dissociative corticosteroid treatment.Frontiers in physiology · 2023Article
- Second Harmonic Generation Morphometry of Muscle Cytoarchitecture in Living Cells.Methods in molecular biology (Clifton, N.J.) · 2023Article
- Myofibrillar Lattice Remodeling Is a Structural Cytoskeletal Predictor of Diaphragm Muscle Weakness in a FibroticInternational journal of molecular sciences · 2022Article
- Absence of the Z-disc protein α-actinin-3 impairs the mechanical stability of Actn3KO mouse fast-twitch muscle fibres without altering their contractile properties or twitch kinetics.Skeletal muscle · 2022Article
- Impact of prolonged sepsis on neural and muscular components of muscle contractions in a mouse model.Journal of cachexia, sarcopenia and muscle · 2021Article
- An advanced optical clearing protocol allows label-free detection of tissue necrosisTheranostics · 2021Article
- Lifespan Analysis of DystrophicFrontiers in physiology · 2021Article
- The unified myofibrillar matrix for force generation in muscle.Nature communications · 2020Article
- Elimination of imaging artifacts in second harmonic generation microscopy using interferometry.Biomedical optics express · 2019Article
- Misplaced Golgi Elements Produce Randomly Oriented Microtubules and Aberrant Cortical Arrays of Microtubules in Dystrophic Skeletal Muscle Fibers.Frontiers in cell and developmental biology · 2019Article
- Optical prediction of single muscle fiber force production using a combined biomechatronics and second harmonic generation imaging approach.Light, science & applications · 2018Article
- Piezo channels and GsMTx4: Two milestones in our understanding of excitatory mechanosensitive channels and their role in pathology.Progress in biophysics and molecular biology · 2017Review
- Early signs of architectural and biomechanical failure in isolated myofibers and immortalized myoblasts from desmin-mutant knock-in mice.Scientific reports · 2017Article
- Altered nuclear dynamics in MDX myofibers.Journal of applied physiology (Bethesda, Md. : 1985) · 2017Article
Corrections and comments
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Authors and funding
10 authors at 4 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Progressive force loss in Duchenne muscular dystrophy is characterized by degeneration/regeneration cycles and fibrosis. Disease progression may involve structural remodeling of muscle tissue. An effect on molecular motorprotein function may also be possible. We used second harmonic generation imaging to reveal vastly altered subcellular sarcomere microarchitecture in intact single dystrophic mdx muscle cells (approximately 1 year old). Myofibril tilting, twisting, and local axis deviations explain at least up to 20% of force drop during unsynchronized contractile activation as judged from cosine angle sums of myofibril orientations within mdx fibers. In contrast, in vitro motility assays showed unaltered sliding velocities of single mdx fiber myosin extracts. Closer quantification of the microarchitecture revealed that dystrophic fibers had significantly more Y-shaped sarcomere irregularities ("verniers") than wild-type fibers (approximately 130/1000 microm(3) vs. approximately 36/1000 microm(3)). In transgenic mini-dystrophin-expressing fibers, ultrastructure was restored (approximately 38/1000 microm(3) counts). We suggest that in aged dystrophic toe muscle, progressive force loss is reflected by a vastly deranged micromorphology that prevents a coordinated and aligned contraction. Second harmonic generation imaging may soon be available in routine clinical diagnostics, and in this work we provide valuable imaging tools to track and quantify ultrastructural worsening in Duchenne muscular dystrophy, and to judge the beneficial effects of possible drug or gene therapies.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.