ArticleInternational journal of molecular medicine2012
Proteomics reveals drastic increase of extracellular matrix proteins collagen and dermatopontin in the aged mdx diaphragm model of Duchenne muscular dystrophy.
Article in International journal of molecular medicine, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 31 papers.
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Who cites it
31 citing papers in PubMed, 60 citations in OpenAlex.
- Dysregulated iron homeostasis in dystrophin-deficient cardiomyocytes: correction by gene editing and pharmacological treatment.Cardiovascular research · 2024Article
- How Can Proteomics Help to Elucidate the Pathophysiological Crosstalk in Muscular Dystrophy and Associated Multi-System Dysfunction?Proteomes · 2024Article
- Extracellular matrix contribution to disease progression and dysfunction in myopathy.American journal of physiology. Cell physiology · 2023Review
- Extracellular Matrix Proteomics: TheBiomolecules · 2023Review
- Myoscaffolds reveal laminin scarring is detrimental for stem cell function while sarcospan induces compensatory fibrosis.NPJ Regenerative medicine · 2023Article
- Multi-omics analysis of sarcospan overexpression in mdx skeletal muscle reveals compensatory remodeling of cytoskeleton-matrix interactions that promote mechanotransduction pathways.Skeletal muscle · 2023Article
- Growth-Promoting Effects of Zhenqi Granules on Finishing Pigs.Animals : an open access journal from MDPI · 2022Article
- Proteomic Identification of Markers of Membrane Repair, Regeneration and Fibrosis in the Aged and Dystrophic Diaphragm.Life (Basel, Switzerland) · 2022Article
- Histopathology of Duchenne muscular dystrophy in correlation with changes in proteomic biomarkers.Histology and histopathology · 2022Review
- Complexity of skeletal muscle degeneration: multi-systems pathophysiology and organ crosstalk in dystrophinopathy.Pflugers Archiv : European journal of physiology · 2021Review
- Extracellular matrix: an important regulator of cell functions and skeletal muscle development.Cell & bioscience · 2021Review
- Impact of estrogen deficiency on diaphragm and leg muscle contractile function in female mdx mice.PloS one · 2021Article
- Blood-derived biomarkers correlate with clinical progression in Duchenne muscular dystrophy.Journal of neuromuscular diseases · 2020Article
- Proteomic profiling of the mouse diaphragm and refined mass spectrometric analysis of the dystrophic phenotype.Journal of muscle research and cell motility · 2019Article
- Proteomic profiling of liver tissue from theClinical proteomics · 2018Article
- Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology · 2017Article
- [Molecular pathogenesis of Duchenne muscular dystrophy-related fibrosis].Der Pathologe · 2017Article
- Spatial and age-related changes in the microstructure of dystrophic and healthy diaphragms.PloS one · 2017Article
- Progressive muscle proteome changes in a clinically relevant pig model of Duchenne muscular dystrophy.Scientific reports · 2016Article
- Elastase levels and activity are increased in dystrophic muscle and impair myoblast cell survival, proliferation and differentiation.Scientific reports · 2016Article
Corrections and comments
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Authors and funding
4 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Duchenne muscular dystrophy is a lethal genetic disease of childhood caused by primary abnormalities in the gene coding for the membrane cytoskeletal protein dystrophin. The mdx mouse is an established animal model of various aspects of X-linked muscular dystrophy and is widely used for studying fundamental mechanisms of dystrophinopathy and testing novel therapeutic approaches to treat one of the most frequent gender-specific diseases in humans. In order to determine global changes in the muscle proteome with the progressive deterioration of mdx tissue with age, we have characterized diaphragm muscle from mdx mice at three ages (8-weeks, 12-months and 22-months) using mass spectrometry-based proteomics. Altered expression levels in diaphragm of 8-week vs. 22-month mice were shown to occur in 11 muscle-associated proteins. Aging in the mdx diaphragm seems to be associated with a drastic increase in the extracellular matrix proteins, collagen and dermatopontin, the molecular chaperone αB-crystallin, and the intermediate filament protein vimentin, suggesting increased accumulation of connective tissue, an enhanced cellular stress response and compensatory stabilization of the weakened membrane cytoskeleton. These proteomic findings establish the aged mdx diaphragm as an excellent model system for studying secondary effects of dystrophin deficiency in skeletal muscle tissue.
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