ArticleMuscle & nerve2021
Muscle architecture is associated with muscle fat replacement in Duchenne and Becker muscular dystrophies.
Article in Muscle & nerve, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers, 1 of them a synthesis that pooled it.
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Who cites it
16 citing papers in PubMed, 1 synthesis or guideline pooled it, 27 citations in OpenAlex.
- X-linked genes influence various complex traits in dairy cattle.BMC genomics · 2023Pooled it
- Trial
- The role of the respiratory physiotherapist in the management of Duchenne muscular dystrophy.Breathe (Sheffield, England) · 2026Review
- Metabolic Complications and Ultrasound-Detected Liver Abnormalities in Paediatric Patients With Duchenne Muscular Dystrophy.Pediatric obesity · 2026Article
- Quantitative MRI Findings and Their Relationship to Muscle Histopathology and Ambulatory Clinical Function in Duchenne Muscular Dystrophy.Journal of cachexia, sarcopenia and muscle · 2026Article
- Investigating the role of EGFR signalling in muscle dystrophies: implications for Duchenne muscular dystrophy.Cell death & disease · 2026Article
- 3D rotational shear wave elasticity imaging (3D-RSWEI) in anisotropic lattice phantoms.Journal of the mechanical behavior of biomedical materials · 2025Article
- Growth differentiation factor 10 inhibits fat infiltration in tongue muscles of mice with high-fat diet.Skeletal muscle · 2025Article
- Longitudinal Follow-Up of Patients With Duchenne Muscular Dystrophy Using QuantitativeJournal of cachexia, sarcopenia and muscle · 2025Article
- Parameter optimization for proton density fat fraction quantification in skeletal muscle tissue at 7 T.Magma (New York, N.Y.) · 2024Article
- Review
- Cellular pathogenesis of Duchenne muscular dystrophy: progressive myofibre degeneration, chronic inflammation, reactive myofibrosis and satellite cell dysfunction.European journal of translational myology · 2023Article
- The metabolomic plasma profile of patients with Duchenne muscular dystrophy: providing new evidence for its pathogenesis.Orphanet journal of rare diseases · 2023Article
- Histopathological correlations and fat replacement imaging patterns in recessive limb-girdle muscular dystrophy type 12.Journal of cachexia, sarcopenia and muscle · 2023Article
- Article
- Muscle architecture is associated with muscle fat replacement in Duchenne and Becker muscular dystrophies.Muscle & nerve · 2021Article
Corrections and comments
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Authors and funding
10 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
INTRODUCTION/
aimsDuchenne and Becker muscular dystrophies (DMD and BMD, respectively) are characterized by fat replacement of different skeletal muscles in a specific temporal order. Given the structural role of dystrophin in skeletal muscle mechanics, muscle architecture could be important in the progressive pathophysiology of muscle degeneration. Therefore, the aim of this study was to assess the role of muscle architecture in the progression of fat replacement in DMD and BMD.
methodsWe assessed the association between literature-based leg muscle architectural characteristics and muscle fat fraction from 22 DMD and 24 BMD patients. Dixon-based magnetic resonance imaging estimates of fat fractions at baseline and 12 (only DMD) and 24 months were related to fiber length and physiological cross-sectional area (PCSA) using age-controlled linear mixed modeling.
resultsDMD and BMD muscles with long fibers and BMD muscles with large PCSAs were associated with increased fat fraction. The effect of fiber length was stronger in muscles with larger PCSA. DISCUSSION: Muscle architecture may explain the pathophysiology of muscle degeneration in dystrophinopathies, in which proximal muscles with a larger mass (fiber length × PCSA) are more susceptible, confirming the clinical observation of a temporal proximal-to-distal progression. These results give more insight into the mechanical role in the pathophysiology of muscular dystrophies. Ultimately, this new information can be used to help support the selection of current and the development of future therapies.
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