ArticlePloS one2013
Changes in muscle cell metabolism and mechanotransduction are associated with myopathic phenotype in a mouse model of collagen VI deficiency.
Article in PloS one, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.
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Who cites it
13 citing papers in PubMed, 30 citations in OpenAlex.
- Effects ofAnimals : an open access journal from MDPI · 2025Article
- Characterization of Proteome Changes in Aged and Collagen VI-Deficient Human Pericyte Cultures.International journal of molecular sciences · 2024Article
- Cartilage-Related Collagens in Osteoarthritis and Rheumatoid Arthritis: From Pathogenesis to Therapeutics.International journal of molecular sciences · 2023Review
- Elucidating Cellular Metabolism and Protein Difference Data from DIGE Proteomics Experiments Using Enzyme Assays.Methods in molecular biology (Clifton, N.J.) · 2023Article
- Molecular Fingerprint of BMD Patients Lacking a Portion in the Rod Domain of Dystrophin.International journal of molecular sciences · 2022Article
- Comparative proteomic analyses of Duchenne muscular dystrophy and Becker muscular dystrophy muscles: changes contributing to preserve muscle function in Becker muscular dystrophy patients.Journal of cachexia, sarcopenia and muscle · 2020Article
- Role of adiponectin in the metabolism of skeletal muscles in collagen VI-related myopathies.Journal of molecular medicine (Berlin, Germany) · 2019Article
- TCA cycle rewiring fosters metabolic adaptation to oxygen restriction in skeletal muscle from rodents and humans.Scientific reports · 2017Article
- Collagen VI Null Mice as a Model for Early Onset Muscle Decline in Aging.Frontiers in molecular neuroscience · 2017Article
- Muscle Interstitial Cells: A Brief Field Guide to Non-satellite Cell Populations in Skeletal Muscle.Methods in molecular biology (Clifton, N.J.) · 2017Review
- Deep RNA profiling identified CLOCK and molecular clock genes as pathophysiological signatures in collagen VI myopathy.Journal of cell science · 2016Article
- Type VI Collagen Regulates Pericellular Matrix Properties, Chondrocyte Swelling, and Mechanotransduction in Mouse Articular Cartilage.Arthritis & rheumatology (Hoboken, N.J.) · 2015Article
- Nutritional status evaluation in patients affected by bethlem myopathy and ullrich congenital muscular dystrophy.Frontiers in aging neuroscience · 2014Article
Corrections and comments
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Authors and funding
10 authors at 7 institutions in 2 countries.
Funding
Abstract
This study identifies metabolic and protein phenotypic alterations in gastrocnemius, tibialis anterior and diaphragm muscles of Col6a1(-/-) mice, a model of human collagen VI myopathies. All three muscles of Col6a1(-/-) mice show some common changes in proteins involved in metabolism, resulting in decreased glycolysis and in changes of the TCA cycle fluxes. These changes lead to a different fate of α-ketoglutarate, with production of anabolic substrates in gastrocnemius and tibialis anterior, and with lipotoxicity in diaphragm. The metabolic changes are associated with changes of proteins involved in mechanotransduction at the myotendineous junction/costameric/sarcomeric level (TN-C, FAK, ROCK1, troponin I fast) and in energy metabolism (aldolase, enolase 3, triose phosphate isomerase, creatine kinase, adenylate kinase 1, parvalbumin, IDH1 and FASN). Together, these change may explain Ca(2+) deregulation, impaired force development, increased muscle-relaxation-time and fiber damage found in the mouse model as well as in patients. The severity of these changes differs in the three muscles (gastrocnemius<tibialis anterior<diaphragm) and correlates to the mass-to-tendon (myotendineous junction) ratio and to muscle morphology.
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Registered trials
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