Characterisation of Progressive Skeletal Muscle Fibrosis in the Mdx Mouse Model of Duchenne Muscular Dystrophy: An In Vivo and In Vitro Study
Abstract
:1. Introduction
2. Results
2.1. Collagen Content, Fibrosis, and Functional Correlations
2.2. Relationship between Muscle Injury and Fibrosis
2.3. Non-Collagen Ground Substance Components of Muscle ECM: GAG/PG Content
2.4. Collagen Turnover Pathways in Primary Muscle Fibroblast Cultures: Collagen Synthesis and Maturation
2.5. Collagen Turnover Pathways in Primary Cell Cultures of Muscle Fibroblasts: Collagen Degradation
3. Discussion
4. Materials and Methods
4.1. Animal Model
4.2. Functional Tests, Collagen Content, and Fibrosis Analysis
4.2.1. Whole-Body Tension Test
4.2.2. Exhaustion Treadmill Test
4.2.3. Histological Analysis and Histochemistry
4.2.4. Sirius Red Staining
4.2.5. Herovici’s Staining
4.3. Analysis of Non-Collagen Ground Substance Components
4.3.1. Alcian Blue Staining
4.3.2. Biglycan Immunohistochemistry
4.4. In Vitro Analysis of Muscle Fibroblasts
4.4.1. Gene Expression Analysis
4.4.2. Slot Blot
4.4.3. SDS-Zymography
4.5. Statistical Analysis
5. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Acknowledgments
Conflicts of Interest
References
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Giovarelli, M.; Arnaboldi, F.; Zecchini, S.; Cornaghi, L.B.; Nava, A.; Sommariva, M.; Clementi, E.G.I.; Gagliano, N. Characterisation of Progressive Skeletal Muscle Fibrosis in the Mdx Mouse Model of Duchenne Muscular Dystrophy: An In Vivo and In Vitro Study. Int. J. Mol. Sci. 2022, 23, 8735. https://doi.org/10.3390/ijms23158735
Giovarelli M, Arnaboldi F, Zecchini S, Cornaghi LB, Nava A, Sommariva M, Clementi EGI, Gagliano N. Characterisation of Progressive Skeletal Muscle Fibrosis in the Mdx Mouse Model of Duchenne Muscular Dystrophy: An In Vivo and In Vitro Study. International Journal of Molecular Sciences. 2022; 23(15):8735. https://doi.org/10.3390/ijms23158735
Chicago/Turabian StyleGiovarelli, Matteo, Francesca Arnaboldi, Silvia Zecchini, Laura Brigida Cornaghi, Ambra Nava, Michele Sommariva, Emilio Giuseppe Ignazio Clementi, and Nicoletta Gagliano. 2022. "Characterisation of Progressive Skeletal Muscle Fibrosis in the Mdx Mouse Model of Duchenne Muscular Dystrophy: An In Vivo and In Vitro Study" International Journal of Molecular Sciences 23, no. 15: 8735. https://doi.org/10.3390/ijms23158735